MedWorm: Ewing's Sarcoma

Evaluation of arsenic trioxide by the pediatric preclinical testing program with a focus on Ewing sarcoma

Pediatric Blood and Cancer — Tue, 07 Feb 2012 05:00:00 +0100

AbstractArsenic trioxide was tested against the PPTP in vitro panel (1.0 nM to 10 µM) and against the PPTP Ewing sarcoma in vivo panel administered intraperitoneally at a dose of 2.5 mg/kg daily × 5 per week for a planned treatment duration of 3 weeks. Arsenic trioxide showed a median relative IC50 value of 0.9 µM, with Ewing sarcoma cell lines having IC50 values similar to those of the remaining PPTP cell lines. Arsenic trioxide did not induce significant differences in EFS distribution compared to control in any of the Ewing sarcoma xenografts studied, and no objective responses were observed. Pediatr Blood Cancer © 2012 Wiley Periodicals, Inc. (Source: Pediatric Blood and Cancer)

MedWorm Sponsor Message: Find the best January Sales in the UK.

Florence and the Machine fan, 20, who died from cancer is immortalised on single cover of her favourite band

the Mail online | Health — Thu, 02 Feb 2012 08:54:16 +0100

A portrait of Natalie Roberts from Alexandria in Scotland, who died from Ewing's Sarcoma in 2010, has been chosen to appear on the record cover by Florence and the Machine. (Source: the Mail online | Health)

Gemcitabine and docetaxel (GEMDOX) for the treatment of relapsed and refractory pediatric sarcomas

Pediatric Blood and Cancer — Thu, 02 Feb 2012 05:00:00 +0100

ConclusionsThe dismal outcomes for patients with relapsed and refractory sarcomas and the lack of effective sarcoma salvage regimens highlight the need for new approaches. This report of the therapeutic activity of gemcitabine and docetaxel (GEMDOX) in rhabdomyosarcoma and other pediatric reports describing activity in osteosarcoma and Ewing sarcoma suggest that this combination should be considered for formal evaluation in a pediatric specific clinical trial. At a minimum, it appears to offer a reasonable, tolerable, palliative option. Pediatr Blood Cancer © 2012 Wiley Periodicals, Inc. (Source: Pediatric Blood and Cancer)

Tumor-specific retargeting of an oncogenic transcription factor chimera results in dysregulation of chromatin and transcription [RESEARCH]

Genome Research — Wed, 01 Feb 2012 05:00:00 +0100

Chromosomal translocations involving transcription factor genes have been identified in an increasingly wide range of cancers. Some translocations can create a protein "chimera" that is composed of parts from different proteins. How such chimeras cause cancer, and why they cause cancer in some cell types but not others, is not understood. One such chimera is EWS–FLI, the most frequently occurring translocation in Ewing Sarcoma, a malignant bone and soft tissue tumor of children and young adults. Using EWS–FLI and its parental transcription factor, FLI1, we created a unique experimental system to address questions regarding the genomic mechanisms by which chimeric transcription factors cause cancer. We found that in tumor cells, EWS–FLI targets regions of the genome distin...

[Primary cervical epidural Ewing sarcoma].

Revista de Neurologia — Wed, 01 Feb 2012 05:00:00 +0100

Authors: Montes-Graciano G, de Quintana-Schmidt C, Clavel-Laria P, Bartumeus-Jene F, Molet-Teixido J PMID: 22278896 [PubMed - in process] (Source: Revista de Neurologia)

Primary malignant tumours of the bone

Surgery (Medicine Publishing) — Mon, 30 Jan 2012 14:21:38 +0100

Abstract: Primary bone tumours are rare, with approximately 400 new cases per year in the UK. The diagnosis of bone tumours are hampered by delays in presentation. Radiographic changes are often characteristic for each type of bone tumour and should alert the physician to investigate further. Investigations should include blood tests, local staging and systemic staging. MRI, CT of the chest and isotope bone scans are important in evaluating the tumour. The most common types of bone tumour are osteosarcoma, chondrosarcoma, Ewing sarcoma, spindle cell sarcoma of bone and chordoma. The important pathological features and treatment of each type of tumour are described in this article. Early contact to a supra-regional bone tumour unit is mandatory when a bone tumour is suspected, where a multi...

MedWorm Sponsor Message: Please support the Doctors In Chains campaign for the medics tortured and sentenced for up to 15 years in Bahrain. #FreeDoctors

Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: Two Unusual Presentations of a Rare Tumor

Diagnostic and Therapeutic Endoscopy — Tue, 24 Jan 2012 11:17:40 +0100

We present here two cases of renal ES/PNET with an uncanny presentation. The first case was discovered after the patient presented clinically with irradiating flank pain, mimicking the pain related with kidney stones. The second case had clinical presentation of pulmonary thromboembolism after the patient was involved in an automobilist accident. The tumors were mainly composed of small blue cells which by immunohistochemical were positive for neural markers, and FISH revealed the translocation 22q12 for the EWSR1 gene. The diagnosis of renal primitive neuroectodermal tumor/EWING tumor is very rare and usually involves several different diagnostic techniques. The differential diagnosis is usually broad with frequent overlapping features between the entities. The cases presented in this pap...

STAT3 Regulates Proliferation and Immunogenicity of the Ewing Family of Tumors In Vitro

Journal of Cancer Epidemiology — Wed, 18 Jan 2012 17:01:46 +0100

The Ewing sarcoma family of tumors (ESFT) represents an aggressive spectrum of malignant tumour types with common defining histological and cytogenetic features. To evaluate the functional activation of signal transducer and activator of transcription 3 (STAT3) in ESFT, we evaluated its activation in primary tissue sections and observed the functional consequences of its inhibition in ESFT cell lines. STAT3 was activated (tyrosine 705-phosphorylated) in 18 out of 31 primary tumours (58%), either diffusely (35%) or focally (23%). STAT3 was constitutively activated in 3 out of 3 ESFT cell lines tested, and its specific chemical inhibition resulted in complete loss of cell viability. STAT3 inhibition in ESFT cell lines was associated with several consistent changes in chemokine profile sugges...

Phase I/II Trial and Pharmacokinetic Study of Cixutumumab in Pediatric Patients With Refractory Solid Tumors and Ewing Sarcoma: A Report From the Children's Oncology Group [Pediatric Oncology]

Journal of Clinical Oncology — Wed, 18 Jan 2012 05:00:00 +0100

Conclusion Cixutumumab is well tolerated in children with refractory solid tumors. The recommended phase II dose is 9 mg/kg. Limited single-agent activity of cixutumumab was seen in ES. (Source: Journal of Clinical Oncology)

Ewing's Sarcoma

About.com Eating Disorders — Mon, 16 Jan 2012 05:00:00 +0100

Ewing's sarcoma is a rare type of bone tumor seen in children and adolescents. Ewing's sarcoma treatment usually consist of chemotherapy, radiation and surgery. (Source: About.com Eating Disorders)

The cell of origin of cranial Ewing's sarcoma: the dilemma persists

Acta Neurochirurgica — Tue, 10 Jan 2012 17:01:17 +0100

Content Type Journal ArticleCategory Letter to the EditorPages 1-2DOI 10.1007/s00701-011-1263-3Authors Pravin Salunke, Department of Neurosurgery, PGIMER, Sector 12, Chandigarh, IndiaKirti Gupta, Department of Histopathology, PGIMER, Chandigarh, IndiaJ Pfeifer, Department of Pathology, Washington University School of Medicine, St Louis, MO, USA Journal Acta NeurochirurgicaOnline ISSN 0942-0940Print ISSN 0001-6268 (Source: Acta Neurochirurgica)

MedWorm Sponsor Message: Find the best January Sales in the UK.

About the article “Primary Ewing’s sarcoma of cranial bones: analysis of ten patients” by Pravin Shashikant Salunke et al. Can we tell the exact origin of cranial Ewing's sarcoma?

Acta Neurochirurgica — Tue, 10 Jan 2012 17:01:16 +0100

Content Type Journal ArticleCategory Letter to the editorPages 1-2DOI 10.1007/s00701-011-1262-4Authors Francesco Portaluri, Department of Neurosurgery, Ospedale San Carlo Borromeo, Milan, ItalyRamon Navarro, Department of Neurosurgery, Stanford University Department of Medicine, 300 Pasteur Dr. R209, Stanford, CA, USA Journal Acta NeurochirurgicaOnline ISSN 0942-0940Print ISSN 0001-6268 (Source: Acta Neurochirurgica)

Rapidly growing intrathoracic extraskeletal Ewing's sarcoma

Interactive CardioVascular and Thoracic Surgery — Mon, 09 Jan 2012 05:00:00 +0100

We report a surgical case of extraskeletal Ewing's sarcoma that had been followed-up as a stable sized tumour for many years, which then grew rapidly within a year. A 27-year old female patient with a rapidly growing abnormal shadow on chest roentgenogram was admitted to our department. She had undergone periodic examinations including chest computed tomography (CT) scans for 6 years since a small nodule in her chest had been pointed out by chest roentgenogram. The initial CT demonstrated a solitary nodule with a diameter of 20 mm on the parietal pleura that covered the V rib of the posterior chest wall. For 5 years the tumour's size did not change noticeably but it suddenly grew to about 90 mm diameter in a year. The tumour volume doubling time was calculated to be 17 days. (Sou...

Acute cauda equina syndrome due to primary Ewing's sarcoma of the spine

Neurology India — Mon, 02 Jan 2012 05:00:00 +0100

R Krishnakumar, J RenjitkumarNeurology India 2011 59(6):931-933 (Source: Neurology India)

Treatment of a severe neglected valgus deformity after excision of the distal fibula for Ewing's sarcoma.

The Journal of Bone and Joint Surgery. British volume — Sun, 01 Jan 2012 05:00:00 +0100

Authors: Jung ST, Park HW, Chung JY Abstract In distal fibular resection without reconstruction, the stabilising effect of the lateral malleolus is lost. Thus, the ankle may collapse into valgus and may be unstable in varus. Here, we describe a child who underwent successful staged surgical correction of a severe neglected valgus deformity after excision of the distal fibula for a Ewing's sarcoma. PMID: 22219262 [PubMed - in process] (Source: The Journal of Bone and Joint Surgery. British volume)

Phase II Study of the Mammalian Target of Rapamycin Inhibitor Ridaforolimus in Patients With Advanced Bone and Soft Tissue Sarcomas [Sarcomas]

Journal of Clinical Oncology — Thu, 29 Dec 2011 05:00:00 +0100

Conclusion Single-agent ridaforolimus in patients with advanced and pretreated sarcomas led to PFS results that compare favorably with historical metrics. A phase III trial based on these data will further define ridaforolimus activity in sarcomas. (Source: Journal of Clinical Oncology)

MedWorm Sponsor Message: Please support the Doctors In Chains campaign for the medics tortured and sentenced for up to 15 years in Bahrain. #FreeDoctors

Tumor necrosis predicts survival following neo‐adjuvant chemotherapy for hepatoblastoma

Pediatric Blood and Cancer — Tue, 20 Dec 2011 05:00:00 +0100

ConclusionsExtent of tumor necrosis following neo‐adjuvant chemotherapy is an independent prognostic factor in patients with newly diagnosed HB. Histological response may potentially be used in strategies to modify post‐surgical therapy to improve survival in HB. Pediatr Blood Cancer © 2011 Wiley Periodicals, Inc. (Source: Pediatric Blood and Cancer)

Initial testing of the multitargeted kinase inhibitor pazopanib by the pediatric preclinical testing program

Pediatric Blood and Cancer — Tue, 20 Dec 2011 05:00:00 +0100

AbstractPazopanib is an oral angiogenesis inhibitor targeting vascular growth factor receptor‐1, ‐2, and ‐3, platelet derived growth factor receptor‐α, platelet derived growth factor receptor‐β, and KIT that has demonstrated activity against a variety of adult cancer xenografts. Pazopanib was tested against a panel of pediatric rhabdomyosarcoma and Ewing sarcoma xenografts at a dose of 108 mg/kg/day or 100 mg/kg twice daily, administered orally for 28 days. While no objective responses were observed, pazopanib induced statistically significant differences in event‐free survival compared to controls in approximately one‐half of the sarcoma xenograft models tested. Though well tolerated, pazopanib showed limited activity against the sarcoma models evaluated, with the best...

Clinical features and outcomes in patients with Ewing sarcoma and regional lymph node involvement

Pediatric Blood and Cancer — Mon, 19 Dec 2011 05:00:00 +0100

ConclusionsPatients with extraskeletal Ewing sarcoma should undergo evaluation for regional node involvement. If validated, our findings indicate that regional node involvement may be an independent adverse prognostic factor in Ewing sarcoma, and potentially useful in risk‐stratifying patients with otherwise localized disease. Pediatr Blood Cancer © 2011 Wiley Periodicals, Inc. (Source: Pediatric Blood and Cancer)

WT1 Regulates VEGF and Hypoxia Response [Cell Biology]

Journal of Biological Chemistry — Fri, 16 Dec 2011 05:00:00 +0100

WT1 is a zinc finger transcription factor expressed at high levels in many types of solid tumors, and high WT1 expression is an adverse prognostic factor. How WT1 contributes to tumor growth and influences prognosis remains unclear. We investigated the hypothesis that WT1 up-regulates VEGF in solid tumors, augmenting the response to hypoxia. We found a correlation between levels of WT1 expression and VEGF expression in Ewing sarcoma cell lines. Transfecting WT1-null SK-ES-1 cells with WT1 up-regulated VEGF mRNA expression and resulted in increased angiogenic activity in vitro. Conversely, diminishing WT1 expression in WT1-positive cell lines using WT1-specific shRNA down-regulated VEGF mRNA expression and decreased angiogenic activity in vitro. Transient transfection assays demonstrated th...

Predictors of acute chemotherapy‐associated toxicity in patients with Ewing sarcoma

Pediatric Blood and Cancer — Fri, 16 Dec 2011 05:00:00 +0100

ConclusionES patients who are younger, of Latino ethnicity, have pelvic tumors or low income have higher rates of toxicity that may require increased supportive care. Treatment on a clinical trial was also associated with higher rates of toxicity, though this finding may reflect better reporting in these patients. Pediatr Blood Cancer © 2011 Wiley Periodicals, Inc. (Source: Pediatric Blood and Cancer)

MedWorm Sponsor Message: Find the best January Sales in the UK.

The contrasting age‐incidence patterns of bone tumours in teenagers and young adults: Implications for aetiology

International Journal of Cancer — Thu, 15 Dec 2011 05:00:00 +0100

AbstractBone tumours comprise 0.2% of cancers overall but 5.7% in 15–24 year‐olds. To explore the relationship with adolescence we have analysed age‐incidence patterns of bone tumours in a large national dataset. Data on incident cases of bone tumours in 0–84 year‐olds in England, 1979‐2003, were extracted from national cancer registration data. Incidence rates per million person‐years by; 5‐year age‐group, sex, morphology and primary site were calculated and adjusted to the world standard population. 9146 cases were identified giving an overall age‐standardized rate of 7.19 per million person‐years. The distribution by morphology was: osteosarcoma, 34.2%; chondrosarcoma, 27.2%; Ewing sarcoma, 19.3%; other, 19.4%. The distribution varied by age. Ewing sarcoma was most...

Pediatric Bone Lesions: Beyond the Plain Radiographic Evaluation

Seminars in Roentgenology — Tue, 13 Dec 2011 21:25:22 +0100

Bone lesions in children are very common and include true bone tumors and tumor-like lesions. More than one-half of all childhood bone neoplasms are benign. The most common benign bone lesions in children are nonossifying fibroma, osteochondroma, cortical desmoid, Langerhans cell histiocytosis, unicameral bone cyst, and aneurysmal bone cyst. The most common malignant bone lesions are osteosarcoma, Ewing sarcoma, and metastatic disease, such as from neuroblastoma. The radiograph remains the cornerstone for evaluation of the pediatric bone lesion. Radiographs provide information on the location of the lesion within the bone, the presence and type of mineralized matrix, the nature of the interface between the tumor and the surrounding host bone, and the reaction of the host bone to the presen...

Nutritional status of children and young adults with Ewing sarcoma or osteosarcoma at diagnosis and during multimodality therapy

Pediatric Blood and Cancer — Tue, 06 Dec 2011 05:00:00 +0100

ConclusionsPediatric patients with Ewing sarcoma or osteosarcoma are at an increased risk for developing malnutrition, in the form of either over‐ or underweight during multimodality therapy. Early recognition of abnormal body mass is required to prevent and to treat long‐term comorbidities caused by malnutrition. Pediatr Blood Cancer © 2011 Wiley Periodicals, Inc. (Source: Pediatric Blood and Cancer)

Oncogene mutation profiling of pediatric solid tumors reveals significant subsets of embryonal rhabdomyosarcoma and neuroblastoma with mutated genes in growth signaling pathways.

Clinical Cancer Research — Mon, 05 Dec 2011 05:00:00 +0100

CONCLUSIONS: In ERMS, ES, and NB, we identified novel occurrences of several oncogene mutations recognized as drivers in other cancers. Overall, NB and ERMS contain significant subsets of cases with non-overlapping mutated genes in growth signaling pathways. Tumor profiling can identify a subset of pediatric solid tumor patients as candidates for kinase inhibitors or RAS-targeted therapies. PMID: 22142829 [PubMed - as supplied by publisher] (Source: Clinical Cancer Research)

High Long-term Local Control with Sacrectomy for Primary High-grade Bone Sarcoma in Children.

Clinical Orthopaedics and Related Research — Sat, 03 Dec 2011 05:00:00 +0100

CONCLUSION: Despite the high incidence of neurologic deficits and complications, sacrectomy achieved local control in patients with high-grade bone sarcomas. Our observations suggest long-term survival and adequate quality of life are possible in the pediatric population. LEVEL OF EVIDENCE: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence. PMID: 22139710 [PubMed - as supplied by publisher] (Source: Clinical Orthopaedics and Related Research)

MedWorm Sponsor Message: Please support the Doctors In Chains campaign for the medics tortured and sentenced for up to 15 years in Bahrain. #FreeDoctors

[Primary malignant bone tumors].

Der Orthopade — Thu, 01 Dec 2011 05:00:00 +0100

Authors: von Eisenhart-Rothe R, Toepfer A, Salzmann M, Schauwecker J, Gollwitzer H, Rechl H Abstract Among human neoplasms, primary malignant bone tumors are fairly rare. They present an incidence rate of roughly 10 cases per 1 million inhabitants per year. During childhood (<15 years), the percentage of malignant bone tumors amounts to 6% of all infantile malignancies. Only leukemia and lymphoma show a higher incidence in adolescence. Of all primary malignant bone tumors, 60% affect patients younger than 45 years and the peak incidence of all bone tumors occurs between 15 and 19 years. The most common primary malignant bone tumors are osteosarcoma (35%), chondrosarcoma (25%), and Ewing's sarcoma (16%). Less frequently (≤ 5%) occurring tumors are chordoma, malignant fibro...

Preliminary Efficacy of the Anti-Insulin-Like Growth Factor Type 1 Receptor Antibody Figitumumab in Patients With Refractory Ewing Sarcoma [Sarcomas]

Journal of Clinical Oncology — Tue, 29 Nov 2011 05:00:00 +0100

Conclusion Figitumumab had modest activity as single agent in advanced ES. A strong association between pretreatment serum IGF-1 and survival benefit was identified. (Source: Journal of Clinical Oncology)

R1507, a Monoclonal Antibody to the Insulin-Like Growth Factor 1 Receptor, in Patients With Recurrent or Refractory Ewing Sarcoma Family of Tumors: Results of a Phase II Sarcoma Alliance for Research Through Collaboration Study [Sarcomas]

Journal of Clinical Oncology — Tue, 29 Nov 2011 05:00:00 +0100

Conclusion R1507 was a well-tolerated agent that had meaningful and durable benefit in a subgroup of patients with ESFT. The identification of markers that are predictive of a benefit is necessary to fully capitalize on this approach. (Source: Journal of Clinical Oncology)

Targeting of Insulin-Like Growth Factor Type 1 Receptor in Ewing Sarcoma: Unfulfilled Promise or a Promising Beginning? [Understanding the Pathway]

Journal of Clinical Oncology — Tue, 29 Nov 2011 05:00:00 +0100

(Source: Journal of Clinical Oncology)

Detection of specific genetic abnormalities by fluorescence in situ hybridization in soft tissue tumors

Pathology International — Mon, 28 Nov 2011 05:00:00 +0100

For the detection of chromosome translocations/chimeric genes and specific genetic abnormalities in soft tissue tumors, we conducted fluorescence in situ hybridization (FISH) analysis on 280 cases of soft tissue and other tumors using formalin‐fixed paraffin‐embedded tissue sections. The detection rate of the FISH split‐signal was 84% (129/154 cases) for the translocation‐associated soft tissue tumors, such as Ewing's sarcoma/primitive neuroectodermal tumor, synovial sarcoma, alveolar rhabdomyosarcoma, myxoid liposarcoma, clear cell sarcoma and so forth. Positive split‐signals from EWSR1, SS18 and FOXO1A probes were detected in 3% (2/64) of various histological types of carcinoma, lymphoma, melanoma, meningioma and soft tissue tumors. In FISH using the INI1/CEP22 probe, the INI1 ...

MedWorm Sponsor Message: Find the best January Sales in the UK.

A to Z of Extraskeletal Ewing Sarcoma Family of Tumors in Adults: Imaging Features of Primary Disease, Metastatic Patterns, and Treatment Responses

American Journal of Roentgenology — Tue, 22 Nov 2011 05:00:00 +0100

CONCLUSION. This article presents the imaging findings of ESFT in the adult, with specific attention to extraskeletal manifestations. (Source: American Journal of Roentgenology)

Primary Cutaneous Ewing's Sarcoma: A Systematic Review Focused on Treatment and Outcome.

The British Journal of Dermatology — Sat, 19 Nov 2011 05:00:00 +0100

Conclusion: This systematic review demonstrated epidemiologic and prognostic differences between Ewing's sarcoma of the bone and primary cutaneous Ewing's sarcoma. Primary cutaneous Ewing's sarcoma has female predominance, occurs at a later age, but, more importantly, has a better outcome. Multimodal therapy for Ewing's sarcoma is associated with immediate and long-term morbidity and mortality. Although the size of our study does not allow definitive conclusion about treatment modalities, we suggest that a less toxic approach compared to conventional treatment should be investigated in primary cutaneous Ewing's sarcoma. PMID: 22098102 [PubMed - as supplied by publisher] (Source: The British Journal of Dermatology)

Testing of the Akt/PKB inhibitor MK‐2206 by the pediatric preclinical testing program

Pediatric Blood and Cancer — Fri, 18 Nov 2011 05:00:00 +0100

ConclusionsMK‐2206 showed its most consistent activity in vitro against ALL cell lines and in vivo against osteosarcoma xenografts. However, no objective responses were observed in solid tumor or ALL xenografts. Further preclinical work evaluating MK‐2206 in pediatric models in the combination therapy setting may contribute to its pediatric development. Pediatr Blood Cancer © 2011 Wiley Periodicals, Inc. (Source: Pediatric Blood and Cancer)

Anti-EGFR antibody cetuximab enhances the cytolytic activity of natural killer cells towards osteosarcoma.

Clinical Cancer Research — Wed, 16 Nov 2011 05:00:00 +0100

CONCLUSION: Our data demonstrate that the cytolytic potential of resting NK cells can be potentiated and directed towards osteosarcoma cells with cetuximab. Therefore, cetuximab-mediated immunotherapy may be considered a novel treatment modality in the management of advanced osteosarcoma. PMID: 22090361 [PubMed - as supplied by publisher] (Source: Clinical Cancer Research)

Tumor-specific retargeting of an oncogenic transcription factor chimera results in dysregulation of chromatin and transcription [RESEARCH]

Genome Research — Tue, 15 Nov 2011 05:00:00 +0100

MedWorm Sponsor Message: Please support the Doctors In Chains campaign for the medics tortured and sentenced for up to 15 years in Bahrain. #FreeDoctors

Diagnostic accuracy of 18 F-FDG-PET and PET/CT in patients with Ewing sarcoma family tumours: a systematic review and a meta-analysis

Skeletal Radiology — Thu, 10 Nov 2011 06:45:40 +0100

Conclusion With regard to the staging and restaging of patients with ESFT, the sensitivity, specificity and accuracy of FDG-PET and PET/CT are high; the combination of FDG-PET or PET/CT with conventional imaging is a valuable tool for the staging and restaging of ESFT and has a relevant impact on the treatment strategy plan. Content Type Journal ArticleCategory Review ArticlePages 1-8DOI 10.1007/s00256-011-1298-9Authors Giorgio Treglia, Department of Bioimaging and Radiological Sciences, Institute of Nuclear Medicine, Catholic University of the Sacred Heart, Rome, ItalyMarco Salsano, Department of Bioimaging and Radiological Sciences, Institute of Nuclear Medicine, Catholic University of the Sacred Heart, Rome, ItalyAntonella Stefanelli, Department of Bioimaging and...

High prevalence of CIC fusion with double‐homeobox (DUX4) transcription factors in EWSR1‐negative undifferentiated small blue round cell sarcomas

Genes, Chromosomes and Cancer — Thu, 10 Nov 2011 05:00:00 +0100

AbstractPrimitive round cell sarcomas of childhood and young adults have been problematic to diagnose and classify. Our goal was to investigate the pathologic and molecular characteristics of small blue round cell tumors (SBRCT) that remained unclassified after exhaustive immunohistochemistry and molecular screening to exclude known sarcoma‐related translocations. As rare examples of EWSR1‐negative SBRCT have been shown to carry rearrangements for FUS and CIC genes, we undertook a systematic screening for these two genes. CIC rearrangements by FISH were detected in 15/22 (68%), while none showed FUS abnormalities. RACE, RT‐PCR, and/or long‐range DNA PCR performed in two cases with frozen material showed that CIC was fused to copies of the DUX4 gene on either 4q35 or 10q26.3. Subseq...

p63 Immunohistochemical Staining Is Limited in Soft Tissue Tumors.

American Journal of Clinical Pathology — Tue, 01 Nov 2011 04:00:00 +0100

Authors: Jo VY, Fletcher CD Abstract p63 is a p53 homolog that is expressed in various normal epithelial tissues and epithelial malignancies. Its expression in mesenchymal lesions has not been examined in depth; therefore, we studied p63 expression by immunohistochemical analysis in 650 soft tissue tumors. We found that p63 expression is limited in soft tissue tumors. The majority of tumors studied were p63-, including all cases of angiosarcoma, lipomatous neoplasms, dermatofibrosarcoma protuberans, solitary fibrous tumor, schwannoma, neurofibroma, gastrointestinal stromal tumor, and leiomyosarcoma. Nuclear p63 reactivity was found in a subset of soft tissue myoepithelioma and myoepithelial carcinoma of soft tissue, cellular neurothekeoma, soft tissue perineurioma, Ewing sarcoma/pe...

Nuclear protein in testis midline carcinomas: a lethal and underrecognized entity.

Archives of Pathology and Laboratory Medicine — Tue, 01 Nov 2011 04:00:00 +0100

Authors: Davis BN, Karabakhtsian RG, Pettigrew AL, Arnold SM, French CA, Musgrave Brill Y Abstract A 54-year-old woman presented with a nasal mass. Biopsy demonstrated undifferentiated tumor cells with extensive apoptosis and necrosis. Chromosome analysis identified a 46,XX,t(15;19)(q13;p13.1) pattern. Nuclear protein in testis (NUT) immunohistochemistry and fluorescence in situ hybridization confirmed NUT rearrangement. A Ewing sarcoma-based chemotherapy regimen and concurrent irradiation obtained a dramatic response; however, the patient died of her disease less than 7 months after initial diagnosis. NUT midline carcinomas are rare, aggressive tumors defined by rearrangement of the NUT gene on 15q14. A solitary translocation involving 15q14 is usually the sole chromosomal abnorm...

Experience with extendable prostheses for malignant bone tumors in children.

J Formos Med Assoc — Tue, 01 Nov 2011 04:00:00 +0100

CONCLUSION: Extendable bone prostheses are useful for reconstruction. According to our experience, this method is indicated in children of at least 10 years of age for whom a future leg-length discrepancy of up to 4 cm is expected and who have a lesion located in the distal femur where the prosthesis can be adequately covered by soft tissue. PMID: 22118316 [PubMed - in process] (Source: J Formos Med Assoc)

MedWorm Sponsor Message: Find the best January Sales in the UK.

Intraoperative microwave inactivation in‐situ of malignant tumors in the scapula

Orthopaedic Surgery — Sun, 23 Oct 2011 05:45:27 +0100

Conclusion: In situ microwave inactivation features simple surgery, reliable effects and patient acceptability, making it an ideal surgical method for malignant tumors in the scapula. (Source: Orthopaedic Surgery)

Radiation recall myositis in pediatric Ewing sarcoma

Pediatric Blood and Cancer — Thu, 20 Oct 2011 04:00:00 +0100

In this report, we present two cases of radiation recall in pediatric Ewing sarcoma patients receiving successive proton radiotherapy and chemotherapy, with magnetic resonance imaging (MRI) of muscle edema within the prior radiation fields. Pediatr Blood Cancer © 2011 Wiley Periodicals, Inc. (Source: Pediatric Blood and Cancer)

Oncogenic ETS proteins mimic activated RAS/MAPK signaling in prostate cells [Research Papers]

Genes and Development — Wed, 19 Oct 2011 04:00:00 +0100

We report that these oncogenic ETS proteins, but not other ETS factors, enhance prostate cell migration. Genome-wide binding analysis matched this specific biological function to occupancy of a unique set of genomic sites highlighted by the presence of ETS- and AP-1-binding sequences. ETS/AP-1-binding sequences are prototypical RAS-responsive elements, but oncogenic ETS proteins activated a RAS/MAPK transcriptional program in the absence of MAPK activation. Thus, overexpression of oncogenic ETS proteins can replace RAS/MAPK pathway activation in prostate cells. The genomic description of this ETS/AP-1-regulated, RAS-responsive, gene expression program provides a resource for understanding the role of these ETS factors in both an oncogenic setting and the developmental processes where these...

Primary intracranial Ewing sarcoma with an unusually aggressive course: A case report and review of the literature

Neuropathology — Tue, 18 Oct 2011 04:00:00 +0100

We present the clinical, radiologic and pathologic findings of an intracranial Ewing sarcoma in a 22‐year‐old woman arising from the dura over the right frontal convexity. The patient underwent craniotomy with gross total excision of the tumor. The tumor showed atypical histology and the diagnosis was confirmed by detection of a rearrangement of the EWSR1 gene by fluorescent in situ hybridization and identification of the diagnostic t(11;22)(q24;q12) translocation by reverse transcription‐polymerase chain reaction. Additional features were detected in this tumor that are known to be associated with an unfavorable prognosis, including loss of p16 expression and gains of chromosomes 1q and 12. The patient experienced the most rapid downhill course reported to date for intracranial Ewin...

Activated growth signaling pathway expression in Ewing sarcoma and clinical outcome

Pediatric Blood and Cancer — Wed, 12 Oct 2011 04:00:00 +0100

ConclusionsIn our series, p‐mTOR and p27KIP1 protein overexpression were independently associated with better survival. Pediatr Blood Cancer © 2011 Wiley Periodicals, Inc. (Source: Pediatric Blood and Cancer)

MedWorm Sponsor Message: Please support the Doctors In Chains campaign for the medics tortured and sentenced for up to 15 years in Bahrain. #FreeDoctors

ASTRO: Ewing Sarcoma Worse in Adults (CME/CE)

MedPage Today Hematology/Oncology — Wed, 05 Oct 2011 16:33:45 +0100

MIAMI BEACH (MedPage Today) -- Survival rates for children with Ewing sarcoma can exceed 75%, but five-year survival for adults with localized disease was just 54%, researchers reported here. (Source: MedPage Today Hematology/Oncology)

Sonography Can Be Useful in Diagnosis of Ewing Sarcoma of the Calcaneus

Journal of Ultrasound in Medicine — Mon, 03 Oct 2011 04:00:00 +0100

(Source: Journal of Ultrasound in Medicine)

Imaging of Thoracic Sarcomas of the Chest Wall, Pleura, and Lung

Seminars in Ultrasound CT and MRI — Sat, 01 Oct 2011 04:00:00 +0100

Primary sarcomas of the thorax are uncommon. The purpose of this review is to describe the radiologic findings of sarcomas affecting the thorax, in particular the chest wall, pleura, and lungs. Most primary sarcomas affecting the thorax arise in the chest wall, and the most common sarcomas of the chest wall are chondrosarcoma, osteosarcoma, Ewing's sarcoma/primitive neuroectodermal tumor, malignant fibrous histiocytoma, and fibrosarcoma. Primary pleural and pulmonary sarcomas are rare. Although histologic analysis is almost always required for accurate diagnosis, imaging is important for staging of these tumors, and several of these tumors have distinctive radiologic features, allowing the radiologist to narrow the differential diagnosis. (Source: Seminars in Ultrasound CT and MRI)

Imaging of Sarcomas of Pelvic Bones

Seminars in Ultrasound CT and MRI — Sat, 01 Oct 2011 04:00:00 +0100

Sarcomas are the most common nonhematologic primary malignancies of bones in the pelvis. Chondrosarcoma, osteosarcoma, and Ewing's sarcoma are the most common sarcomas to originate from the pelvic bones. Various imaging modalities such as magnetic resonance imaging and computed tomography play an important role in the detection, characterization, and staging of these lesions. Biopsy, usually performed with imaging guidance, is essential for the histologic diagnosis of these tumors and for planning therapeutic options. Despite considerable advances in treatment options, sarcomas in the pelvic bones generally are associated with poorer outcomes than sarcomas in the appendicular skeleton because of the larger size of the lesions at the time of discovery and the difficulty of obtaining a wide ...

Pediatric Soft Tissue Sarcomas

Seminars in Ultrasound CT and MRI — Sat, 01 Oct 2011 04:00:00 +0100

After a brief discussion of the rarity of soft tissue sarcomas in children and of the limited ability of magnetic resonance imaging to provide a tissue diagnosis, this article discusses the incidence, presentation, treatment, prognosis, and imaging characteristics of the more common and unusual pediatric soft tissue sarcomas. It begins with extensive discussion of rhabdomyosarcoma, synovial sarcoma, and congenital/infantile fibrosarcoma. It then presents a more abbreviated discussion of uncommon tumors such as alveolar soft part sarcoma, epithelioid sarcoma, extraosseous Ewing's sarcoma, granulocytic sarcoma, hemangiopericytoma, liposarcoma, malignant fibrous histiocytoma, malignant peripheral nerve sheath tumor, and undifferentiated sarcoma. (Source: Seminars in Ultrasound CT and MRI)

MedWorm Sponsor Message: Find the best January Sales in the UK.

Ewing sarcoma: an unusual case in the inferior jaw

International Journal of Oral and Maxillofacial Surgery — Sat, 01 Oct 2011 04:00:00 +0100

Ewing's sarcoma accounts for 4–6% of primary malignant bone tumors and it affects the head and neck in only 1–4% cases. It is a rare undifferentiated, round and small-cells tumor of uncertain histogenesis. A 16-year-old female patient presented extrusion, accentuated mobility and slightly sintomatology at 36 tooth. Radiographically radiolucent round areas involving the apical region of 36 tooth were observed. The CBCT exam showed hypodense area with destruction of lingual plate nearby to the 36 tooth. Exploratory surgery was performed with lingual access discovering an empty cavity. Twenty days afterwards, the patient returned without mobility at the teeth, suggesting the diagnostic of Simple Bone Cyst. After thirty days, the patient presented vestibular growth, pain, considerable dent...

Congenital extraskeletal Ewing's sarcoma of chest wall - A rare case report.

Indian Journal of Pathology and Microbiology — Sat, 01 Oct 2011 04:00:00 +0100

We report a case of extraskeletal congenital Ewing's sarcoma in a female fetus delivered at 34 weeks of gestation who died immediately after birth. In English literature, majority of cases of Ewing's sarcoma in neonates reported were skeletal. To the best of our knowledge, very few cases of extra-skeletal Ewing's sarcoma in neonates are reported in the literature. PMID: 22234116 [PubMed - in process] (Source: Indian Journal of Pathology and Microbiology)

miR‐34a predicts survival of Ewing's sarcoma patients and directly influences cell chemosensitivity and malignancy

The Journal of Pathology — Thu, 29 Sep 2011 04:00:00 +0100

In this study, we analyzed the miRNAs discriminating Ewing's sarcoma (EWS) patients with different clinical outcome in order to identify new indicators of prognosis. miRNA expression was investigated in 49 primary EWS by using the Agilent Human miRNA microarray v.2 and/or qRT‐PCR. Statistical power of samples studied for miRNA expression was verified, indicating adequate sample size. Microarray analysis defined a signature of 5 miRNAs (miR‐34a, miR‐23a, miR‐92a, miR‐490‐3p, and miR‐130b) as an independent predictor of risk to disease progression and survival. Validation analysis in the extended samples set indicated that both miR‐34a and miR490‐3p achieved sufficient statistical power to predict prognosis. Results were particularly robust for miR‐34a, which appeared ass...

Chest Wall Ewing Sarcoma Presenting as Abdominal Pain in a 12-Year-Old Boy

The Journal of Pediatrics — Thu, 29 Sep 2011 04:00:00 +0100

A 12-year-old boy presented with a 3-month history of right upper quadrant abdominal pain, a 15%-20% loss of body weight, and a 3-week history of cough and dyspnea. He did not have jaundice, fever, acholic or melanotic stools, or nausea/vomiting. Respirations were 40/minute and regular, and heart rate was 125 beats/minute. Heart sounds and point of maximum impulse were displaced to the left. He exhibited increased work of breathing, with absent breath sounds on the right side and diffuse crackles on the left side. Examination revealed a palpable 10 × 8 cm, firm, immobile, nontender subcutaneous mass protruding from the right side of the back. Laboratory studies were significant only for mild normocytic anemia (hemoglobin, 9.4 g/dL, mean corpuscular volume, 75 fL) and mildly elevated serum...

Ewing's sarcoma metastasis into the breast.

Diagnostic and Interventional Radiology : The Turkish Society of Radiology — Tue, 27 Sep 2011 04:00:00 +0100

Authors: Orguc S, Basara I, Pocan T, Ozguven AA, Ozkol M Abstract The metastasis of extramammary malignancies into the breast is very unusual. Lymphoma, malignant melanoma, and rhabdomyosarcoma are the most common tumors that metastasize into breast tissue. The histological spectrum of breast masses in children and adolescents is different from that of adults. Imaging findings are useful for performing a diagnosis, but in a patient with a known malignancy, any enlarging breast mass, even one with a benign radiological appearance, should be investigated with a biopsy. In this article, we present the imaging findings of a 12-year-old female patient with breast metastasis of Ewing's sarcoma. PMID: 21948698 [PubMed - as supplied by publisher] (Source: Diagnostic and Interventional ...

MedWorm Sponsor Message: Please support the Doctors In Chains campaign for the medics tortured and sentenced for up to 15 years in Bahrain. #FreeDoctors

Novel peptide binds EWS-FLI1 and reduces the oncogenic potential in Ewing tumors.

Cell Cycle — Fri, 23 Sep 2011 18:54:43 +0100

Authors: Erkizan HV, Scher LJ, Gamble SE, Barber-Rotenberg JS, Sajwan KP, Uren A, Toretsky JA Abstract Ewing tumor is driven by the oncogenic EWS-FLI1 fusion protein that functions as an aberrant transcription factor. The identification of EWS-FLI1 protein partners is essential to enhance its vulnerability as a therapeutic target. We utilized phage display library screening against recombinant EWS-FLI1 protein. We identified 27 unique Ewing sarcoma binding peptides. The cytotoxicity evaluation of these peptides with in EWS-FLI1 containing cell lines yielded one potent peptide called ESAP1 (TMRGKKKRTRAN). ESAP1 binds EWS-FLI1 with 0.202 micromolar affinity as measured in surface plasmon resonance. The minimal interaction region of ESAP1 is characterized and found that the lysine res...

An Incidentaloma: Primitive Neuroectodermal Tumor of the Thymus

Infectious Diseases in Obstetrics and Gynecology — Thu, 22 Sep 2011 16:26:01 +0100

After presenting for a routine screening exam, and 57-year-old man was diagnosed with an incidentaloma—a primitive neuroectodermal tumor (PNET) of the thymus. A member of the Ewing sarcoma family of tumors, a PNET is typically regarded as a malignancy of childhood and adolescence, usually occurring in the central nervous system. In the case at hand, our patient had an extremely unusual presentation, given his age and tumor location. Initial presentation is the only predictor for long-term survival. Current treatment recommendations advocate complete surgical resection whenever possible, radiation therapy, and adjuvant versus neoadjuvant chemotherapy. (Source: Infectious Diseases in Obstetrics and Gynecology)

From conventional fluid cytology to unusual histological diagnosis: Report of four cases

Diagnostic Cytopathology — Mon, 19 Sep 2011 04:00:00 +0100

AbstractMalignant small round cell tumors represent a diagnostic challenge for cytologists and pathologists. This case series describes four cases of unusual metastasis of small round cell tumors subtypes into body cavities generating effusions in which fluid cytological examination suggested the neuroendocrine origin of the tumors. Tumor diagnosis (Ewing sarcoma/primitive neuroectodermal tumor and desmoplastic small round cell tumors) were unknown at the cytological evaluation. We can highlight the importance of the accurate analyses of body fluids, both for early diagnosis of metastatic disease, and for the diagnosis of primary tumor when serous effusion is the first manifestation of the neoplasia. Diagn. Cytopathol. 2011; © 2011 Wiley‐Liss, Inc. (Source: Diagnostic Cytopathology)

Treatment of pelvic defect and infection with endoprosthesis exposure by topical negative pressure and irrigation with myocutaneous flap

Microsurgery — Wed, 14 Sep 2011 04:00:00 +0100

We describe a 17‐year‐old woman with Ewing sarcoma in the pelvis who underwent hemipelvectomy and reconstruction with an artificial hip joint and bone cement. After the operation, skin necrosis and deep infection with methicillin‐resistant Staphylococcus aureus (MRSA) were observed. Debridement resulted in exposure of the artificial joint and bone cement. Topical negative pressure (TNP) and irrigation successfully eradicated the infection. The skin and soft‐tissue defect was subsequently reconstructed using a combination of free latissimus dorsi myocutaneous flap and serratus anterior muscle flap. To our knowledge, this is the first described case of combined TNP and irrigation with myocutaneous flap for the treatment of pelvic infection and skin and soft‐tissue defect with endop...

Incidence and survival of malignant bone sarcomas in England 1979‐2007

International Journal of Cancer — Mon, 12 Sep 2011 17:51:32 +0100

AbstractPrimary malignant bone sarcomas (MBS) are rare and there are few studies examining their incidence and outcome. Here, the incidence and survival of all subtypes of MBS registered in England between 1979 and 2007 were analysed from patient registry data held by the National Cancer Intelligence Network (NCIN). Over 11,002 new cases of MBS were registered, an average of 379 per year. There was no change in incidence demonstrated over the study period (p=0.08). Although a peak incidence is observed in adolescence, approximately half of MBS are diagnosed in patients over 50 years. An improvement in outcome of MBS was observed between those patients registered from 1979‐1983 and 1983‐1987 (p<0.0001), but there has been no improvement since. In the most recent period studied (patie...

MedWorm Sponsor Message: Find the best January Sales in the UK.

Incidence and survival of malignant bone sarcomas in England 1979–2007

International Journal of Cancer — Mon, 12 Sep 2011 04:00:00 +0100

AbstractPrimary malignant bone sarcomas (MBS) are rare and there are few studies examining their incidence and outcome. Here, the incidence and survival of all subtypes of MBS registered in England between 1979 and 2007 were analysed from patient registry data held by the National Cancer Intelligence Network (NCIN). Over 11,002 new cases of MBS were registered, an average of 379 per year. There was no change in incidence demonstrated over the study period (p = 0.08). Although a peak incidence is observed in adolescence, approximately half of MBS are diagnosed in patients over 50 years. An improvement in outcome of MBS was observed between those patients registered from 1979 to 1983 and 1983 to 1987 (p < 0.0001), but there has been no improvement since. In the most recent period studied ...

Ewing's sarcoma of the orbit with intracranial extension: A rare cause of unilateral proptosis

Journal of Pediatric Neurosciences — Thu, 01 Sep 2011 23:00:00 +0100

Anup P Nair, Guruprasad Bettaswamy, Awdhesh K Jaiswal, Pallav Garg, Sushila Jaiswal, Sanjay BehariJournal of Pediatric Neurosciences 2011 6(1):36-39Ewing's sarcoma causing unilateral proptosis along with bifrontal extradural infiltration in a child is an unusual presentation. A female patient presented with features of painless proptosis of the left eye with visual deterioration. Her radiology revealed an infiltrating intraorbital, extraconal tumor with intracranial bifrontal extradural extension causing mass effect. Total excision of the intraorbital and intracranial part of the tumor along with postoperative chemo- and radiotherapy brought about a substantial relief. The clinicoradiological presentation and management of this rare entity are discussed. (Source: Journal of Pediatri...

Long-term IL-2 therapy after transplantation of T cell depleted stem cells from alternative donors in children

Best Practice and Research. Clinical Haematology — Thu, 01 Sep 2011 04:00:00 +0100

In conclusion, long-term low-dose IL-2 subcutaneous application is feasible in children due to a low side effect profile even after HLA mismatched transplantation and may be a strategy to prevent relapse in pediatric malignancies with extremely high risk of relapse. (Source: Best Practice and Research. Clinical Haematology)

Primitive Round Cell Neoplasms

Surgical Pathology Clinics — Thu, 01 Sep 2011 04:00:00 +0100

Abstract: Primitive round cell neoplasms (small round cell tumors) of soft tissue are a diverse group of malignant tumors composed of monotonous undifferentiated cells with high nuclear-cytoplasmic ratio. Many occur more frequently, although not exclusively, in childhood. As tumors with primitive round cell morphology are seen in virtually every basic tumor category, the diagnosis of small round cell neoplasms requires the use of ancillary diagnostic techniques: immunohistochemistry and often molecular genetics. The principal tumors in this group include Ewing sarcoma/primitive neuroectodermal tumor, desmoplastic small round cell tumor, alveolar rhabdomyosarcoma, poorly differentiated synovial sarcoma, neuroblastoma, and ganglioneuroblastoma. (Source: Surgical Pathology Clinics)

A novel EWSR1‐CREB3L1 fusion transcript in a case of small cell osteosarcoma

Genes, Chromosomes and Cancer — Sat, 27 Aug 2011 12:57:37 +0100

We report the case of a 12‐year‐old girl with multifocal small cell osteosarcoma positive for the Ewing sarcoma breakpoint region 1 (EWSR1) gene rearrangement by interphase fluorescent in situ hybridization and negative for EWSR1‐FLI1, EWSR1‐ERG, and EWSR1‐WT1 fusion transcripts by reverse transcriptase PCR. Rapid amplification of cDNA ends revealed exon 6 of the cAMP‐responsive element binding protein 3‐like 1 gene (CREB3L1, also known as “OASIS,” NM_52854.2) fused in‐frame to the EWSR1 exon 11, consistent with the EWSR1‐CREB3L1 fusion transcript expressed in tumor tissue. The corresponding chimeric gene was confirmed by amplification and subsequent sequencing of the genomic breakpoint between introns 11 and 5 of EWSR1 and CREB3L1, respectively. An ∼70 kDa product ...

MedWorm Sponsor Message: Please support the Doctors In Chains campaign for the medics tortured and sentenced for up to 15 years in Bahrain. #FreeDoctors

CD86 +1057G/A Polymorphism and Susceptibility to Ewing's Sarcoma: A Case–Control Study

DNA and Cell Biology — Fri, 26 Aug 2011 20:04:39 +0100

DNA and Cell Biology , Vol. 0, No. 0. (Source: DNA and Cell Biology)

CAPER‐α alternative splicing regulates the expression of vascular endothelial growth factor165 in ewing sarcoma cells

Cancer — Wed, 24 Aug 2011 23:00:00 +0100

CONCLUSIONS:Increased VEGF165 expression is secondary to the down‐regulation of CAPER‐α by EWS/FLI‐1. CAPER‐α mediates alternative splicing and controls the shift from VEGF189 to VEGF165. Cancer 2011;. © 2011 American Cancer Society. (Source: Cancer)

Preclinical evaluation of pemetrexed in pediatric solid tumors

Pediatric Blood and Cancer — Tue, 16 Aug 2011 23:00:00 +0100

AbstractRenewed interest in antifols for the treatment of childhood cancers has resulted from identification of novel antifols with broad spectrums of anti‐cancer activity. We evaluated the in vitro cytotoxicity of methotrexate and pemetrexed in a panel of 12 pediatric solid tumor cell lines using the sulforhodamine‐B assay. The Ewing sarcoma (ES) cell lines demonstrated the greatest sensitivity to both methotrexate and pemetrexed. Expression of folate pathway genes (DHFR, TS, GARFT, GGH) did not correlate with in vitro drug sensitivity. Further evaluation of pemetrexed for the treatment of ES is warranted. Pediatr Blood Cancer © 2011 Wiley‐Liss, Inc. (Source: Pediatric Blood and Cancer)

Anterior spinal artery syndrome after percutaneous vertebroplasty

The Spine Journal — Thu, 11 Aug 2011 04:00:00 +0100

Conclusions: Although percutaneous vertebroplasty has many benefits, including its simplicity and relative safety, it could lead to serious complications. The current case demonstrates the direct leakage of cement within the anterior spinal artery leading to an irreversible paralysis. The clinicians should be aware of such complications to happen and explain it to their patients. (Source: The Spine Journal)

MedWorm Sponsor Message: Find the best January Sales in the UK.

1q gain and CDT2 overexpression underlie an aggressive and highly proliferative form of Ewing sarcoma

Oncogene — Sun, 07 Aug 2011 23:00:00 +0100

Authors: C Mackintosh, J L Ordóñez, D J García-Domínguez, V Sevillano, A Llombart-Bosch, K Szuhai, K Scotlandi, M Alberghini, R Sciot, F Sinnaeve, P C W Hogendoorn, P Picci, S Knuutila, U Dirksen, M Debiec-Rychter, K-L Schaefer & E de Álava (Source: Oncogene)

The Ewing Sarcoma Protein Regulates DNA Damage-Induced Alternative Splicing

Molecular Cell — Fri, 05 Aug 2011 00:00:00 +0100

We report that EWS depletion results in altern.... (Source: Molecular Cell)

A Phase I, Pharmacokinetic and Pharmacodynamic Study of Dalotuzumab (MK-0646), an Anti-IGF-1R Monoclonal Antibody, in Patients with Advanced Solid Tumors.

Clinical Cancer Research — Mon, 01 Aug 2011 23:00:00 +0100

CONCLUSIONS: Dalotuzumab was generally well-tolerated, exhibited dose-proportional PK, inhibited IGF-1R pathway signaling and cell proliferation in treated tumors, and demonstrated clinical activity. The low clearance rate and long terminal half-life support more extended dosing intervals. PMID: 21810918 [PubMed - as supplied by publisher] (Source: Clinical Cancer Research)

Molecular characterization of an EWSR1–POU5F1 fusion associated with a t(6;22) in an undifferentiated soft tissue sarcoma

Cancer Genetics and Cytogenetics — Mon, 01 Aug 2011 04:00:00 +0100

This report is novel in that to our knowledge, it is the first complete molecular characterization of an EWSR1–POU5F1 fusion in a soft tissue sarcoma. Evaluation of existing data on the known EWSR1–POU5F1 tumors suggests that the fusion gene functions in a wide variety of cell types and may modify the differentiation state of cells, resulting in susceptibility to tumorigenesis. (Source: Cancer Genetics and Cytogenetics)

Vaginal metastasis of a Ewing Sarcoma five years after resection of the primary tumor.

BioMed Central — Sun, 31 Jul 2011 23:00:00 +0100

${item.shortDescription} (Source: BioMed Central)

MedWorm Sponsor Message: Please support the Doctors In Chains campaign for the medics tortured and sentenced for up to 15 years in Bahrain. #FreeDoctors

Primary metastasized extraskeletal Ewing sarcoma of the vulva: report of a case and review of the literature

Archives of Gynecology and Obstetrics — Fri, 29 Jul 2011 16:06:33 +0100

Conclusion The treatment of the Ewing’s sarcomas and PNETs requires a multidisciplinary systemic approach. Despite its rarity, the differential diagnosis of vulvar sarcoma must be considered in young women. Content Type Journal ArticlePages 1-5DOI 10.1007/s00404-011-2011-xAuthors Katharina Kelling, Department of Obstetrics and Gynecology, University Hospital of Schleswig-Holstein, Campus Luebeck, Ratzeburger Allee 160, 23538 Luebeck, GermanyFrank Noack, Department of Pathology, University Hospital of Schleswig-Holstein, Campus Luebeck, Luebeck, GermanyChristopher Altgassen, Department of Obstetrics and Gynecology, University Hospital of Schleswig-Holstein, Campus Luebeck, Ratzeburger Allee 160, 23538 Luebeck, GermanyPeter Kujath, Department of Surgery, University H...

Neuropeptide Y and Dipeptidyl Peptidases in Ewing Sarcoma [Cell Biology]

Journal of Biological Chemistry — Thu, 28 Jul 2011 23:00:00 +0100

In conclusion, DPPs act as survival factors for ESFT cells and protect them from cell death induced by endogenous NPY. This is the first demonstration that intracellular DPPs are involved in regulation of ESFT growth and may become potential therapeutic targets for these tumors. (Source: Journal of Biological Chemistry)

Evaluation of polymorphisms in EWSR1 and risk of Ewing sarcoma: A report from the childhood cancer survivor study

Pediatric Blood and Cancer — Sun, 24 Jul 2011 23:00:00 +0100

ConclusionsVariations in EWSR1 at known SNPs or across intron 7 are not associated with the diagnosis of Ewing sarcoma. EWSR1 does not appear to be an Ewing sarcoma susceptibility gene. The genetic basis for this disease remains unknown. Pediatr Blood Cancer © 2011 Wiley‐Liss, Inc. (Source: Pediatric Blood and Cancer)

Askin's Tumor: A Dual Case Study

Anesthesiology Research and Practice — Tue, 19 Jul 2011 14:47:07 +0100

Askin's tumor is a rare tumor arising from the chest wall. It is a subset of Ewing sarcoma characterized histologically by the presence of small round blue cells. It is a highly malignant tumor with guarded prognosis, which is dependent upon the extension of tumor at the time of diagnosis. A dual paper of Askin's tumors in young boys is being presented here. (Source: Anesthesiology Research and Practice)

Radiology-pathology conference: primary perinephric and renal extraosseous Ewing's sarcoma

Clinical Imaging — Mon, 18 Jul 2011 04:00:00 +0100

We present a 38-year-old man with primary Ewing's sarcoma of the perinephric fat and kidney diagnosed 2 years earlier who subsequently developed an infiltrating left renal and perinephric mass. (Source: Clinical Imaging)

MedWorm Sponsor Message: Find the best January Sales in the UK.

Macrophage Infiltration Predicts a Poor Prognosis for the Human Ewing Sarcoma.

The American Journal of Pathology — Fri, 15 Jul 2011 23:00:00 +0100

The objective of the present study was to investigate whether tumor-associated macrophages (TAMs) affect the development of EWS. TAMs were isolated from mouse xenografts using CD11b magnetic beads and examined for their cytokine expression and osteoclastic differentiation. To evaluate the role of TAMs in xenograft formation, liposome-encapsulated clodronate was used to deplete TAMs in mice. Macrophage infiltration and tumor microvascular density were histologically evaluated in 41 patients with EWS, and association with prognosis was examined using Kaplan-Meier survival analysis. In mouse EWS xenografts, TAMs expressed higher concentrations of cytokines including interleukin-6, keratinocyte-derived chemokine, and monocyte chemotactic protein-1. TAMs were more capable than normal monocytes ...

Notch signaling is off and is uncoupled from HES1 expression in Ewing's sarcoma

The Journal of Pathology — Thu, 14 Jul 2011 23:00:00 +0100

AbstractNotch can act as an oncogene or as a tumor suppressor and thus can either promote or inhibit tumor cell growth. To establish Notch status in Ewing's sarcoma family of tumors (ESFT), we investigated the Notch pathway by gene expression profiling meta‐analysis or immunohistochemistry in samples obtained from 96 and 24 ESFT patients, respectively. We find that although Notch receptors were highly expressed, Notch did not appear to be active as evidenced by the absence of Notch receptors in cell nuclei. In contrast, we show that Notch receptors known to be active in colon adenocarcinoma, hepatocarcinoma, and pancreatic carcinoma stain cell nuclei in these tumors. High expression of the Notch effector HES1 transcription factor, usually used as a surrogate marker for active Notch, was ...

Notch signalling is off and is uncoupled from HES1 expression in Ewing's sarcoma

The Journal of Pathology — Thu, 14 Jul 2011 23:00:00 +0100

AbstractNotch can act as an oncogene or as a tumour suppressor and thus can either promote or inhibit tumour cell growth. To establish Notch status in Ewing's sarcoma family of tumours (ESFT), we investigated the Notch pathway by gene expression profiling meta‐analysis or immunohistochemistry in samples obtained from 96 and 24 ESFT patients, respectively. We found that although Notch receptors were highly expressed, Notch did not appear to be active, as evidenced by the absence of Notch receptors in cell nuclei. In contrast, we show that Notch receptors known to be active in colon adenocarcinoma, hepatocarcinoma, and pancreatic carcinoma stain cell nuclei in these tumours. High expression of the Notch effector HES1 transcription factor, usually used as a surrogate marker for active Notch...

Phase I Trial of Cixutumumab Combined with Temsirolimus in Patients with Advanced Cancer.

Clinical Cancer Research — Mon, 11 Jul 2011 23:00:00 +0100

CONCLUSIONS: Temsirolimus combined with cixutumumab was well tolerated. We are currently enrolling expansion cohorts at the MTD for Ewing's sarcoma and adrenocortical carcinoma. PMID: 21750201 [PubMed - as supplied by publisher] (Source: Clinical Cancer Research)

Tumoral thrombosis of inferior vena cava and iliac veins resulting in unilateral lower limb edema in a young adult: A rare presentation of skeletal Ewing's sarcoma

European Journal of Radiology Extra — Sun, 03 Jul 2011 23:00:00 +0100

Abstract: Association of venous thromboembolic events (TEs) with malignancy is well known. Ewing's sarcoma usually presents as swelling at the primary site, however presenting as unilateral lower limb edema due to tumoral thrombosis of right iliac vein and IVC is a rare event. A 30-year-old male patient with extensive right lower limb swelling was admitted and imaging studies (plain film, ultrasonography, Doppler, computed tomography) revealed a mass arising from right iliac bone, adjoining sacrum and tumoral thrombosis of right iliac veins extending into inferior vena cava. Histopathological diagnosis was Ewing's sarcoma. Patient was treated successfully with chemotherapy and surgical embolectomy. So a young patient presenting with deep vein thrombosis should be investigated not only to e...

MedWorm Sponsor Message: Please support the Doctors In Chains campaign for the medics tortured and sentenced for up to 15 years in Bahrain. #FreeDoctors

Targeting The Ewing Sarcoma Family Of Tumors

Health News from Medical News Today — Sat, 02 Jul 2011 07:00:00 +0100

The Max Cure Foundation and the Samuel Waxman Cancer Research Foundation have partnered to establish a fund in pediatric cancer research. With that goal in mind, the two Foundations are proud to announce the award of $100,000 to Erwin G. Van Meir, Ph.D., of Emory University School of Medicine in Atlanta. Van Meir's research aims to reprogram Ewing sarcoma using a small molecule that will target the Ewing sarcoma family of tumors (ESFT) oncoprotein. Ewing sarcoma, a type of cancer that forms in the bone or soft tissue, affects primarily young children and adolescents... (Source: Health News from Medical News Today)

Targeting the Ewing sarcoma family of tumors

EurekAlert! - Social and Behavioral Science — Fri, 01 Jul 2011 04:00:00 +0100

(Samuel Waxman Cancer Research Foundation) The Max Cure Foundation and the Samuel Waxman Cancer Research Foundation award $100,000 to Erwin G. Van Meir, Ph.D., of Emory University School of Medicine to further his research in Ewing sarcoma. (Source: EurekAlert! - Social and Behavioral Science)

Imaging pediatric bone sarcomas.

Radiologic Clinics of North America — Thu, 30 Jun 2011 23:00:00 +0100

This article reviews the 3 most common pediatric bone sarcomas-osteosarcoma, Ewing sarcoma, and chondrosarcoma-and their imaging as applicable to contemporary disease staging and monitoring, and explores the roles of evolving imaging techniques. PMID: 21807172 [PubMed - in process] (Source: Radiologic Clinics of North America)

Promiscuous partnerships in Ewing's sarcoma

Cancer Genetics and Cytogenetics — Thu, 30 Jun 2011 23:00:00 +0100

Ewing’s sarcoma is a highly aggressive bone and soft tissue tumor of children and young adults. At the molecular genetic level Ewing’s sarcoma is characterized by a balanced reciprocal translocation, t(11;22)(q24;q12), which encodes an oncogenic fusion protein and transcription factor EWS/FLI. This tumor-specific chimeric fusion retains the amino terminus of EWS, a member of the TET (TLS/EWS/TAF15) family of RNA-binding proteins, and the carboxy terminus of FLI, a member of the ETS family of transcription factors. In addition to EWS/FLI, variant translocation fusions belonging to the TET/ETS family have been identified in Ewing’s sarcoma. These studies solidified the importance of TET/ETS fusions in the pathogenesis of Ewing’s sarcoma and have since been used as diagnostic markers ...

Surgical treatment of patients with disseminated Ewing sarcoma in our clinical experience.

Ortopedia, Traumatologia, Rehabilitacja — Wed, 29 Jun 2011 23:00:00 +0100

Conclusions: Surgery appears to prolong survival among patients with disseminated Ewing's sarcoma. <br /> PMID: 21750357 [PubMed - in process] (Source: Ortopedia, Traumatologia, Rehabilitacja)

MedWorm Sponsor Message: Find the best January Sales in the UK.

The Use of Veritas Collagen Matrix to Reconstruct the Posterior Chest Wall After Costovertebrectomy [HOW TO DO IT]

The Annals of Thoracic Surgery — Sun, 26 Jun 2011 23:00:00 +0100

We describe the reconstruction of the chest wall with Veritas (Synovis, St Paul, MN) collagen matrix of a posterior chest wall defect after costovertebrectomy for Ewing's sarcoma. En bloc resection was performed, including partial D7 through D9 vertebrectomy along with the posterolateral segments of corresponding ribs. The collagen matrix patch was sutured to the spine stabilizer and the surrounding rib segments and was covered by previously raised latissimus dorsi and trapezius muscle flaps. Excellent stabilization was obtained. (Source: The Annals of Thoracic Surgery)

EWS/FLI Target Gene Regulation Mechanisms [Gene Regulation]

Journal of Biological Chemistry — Thu, 23 Jun 2011 23:00:00 +0100

In this study, we investigated the specific processes EWS/FLI1 utilizes to alter gene expression. Using both heterologous NIH 3T3 and human Ewing Family Tumor cell lines, we have demonstrated by quantitative pre-mRNA analysis that EWS/FLI1 repressed the expression of previously validated direct target genes at the level of transcript synthesis. ChIP experiments showed that EWS/FLI1 decreases the amount of Pol II at the promoter of down-regulated genes in both murine and human model systems. However, in down-regulated target genes, there was a significant disparity between the modulation of cognate mRNA and pre-mRNAs, suggesting that these genes could also be regulated at a posttranscriptional level. Confirming this, we found that EWS/FLI1 decreased the transcript half-life of insulin-like ...

Identification of an Inhibitor of the EWS-FLI1 Oncogenic Transcription Factor by High-Throughput Screening

JNCI — Mon, 20 Jun 2011 23:00:00 +0100

Conclusion Mithramycin inhibits EWS-FLI1 activity and demonstrates ESFT antitumor activity both in vitro and in vivo. (Source: JNCI)

Sarcomas gone bad: What to do about recurrent Ewing sarcoma

Pediatric Blood and Cancer — Wed, 15 Jun 2011 23:00:00 +0100

(Source: Pediatric Blood and Cancer)

Ewing sarcoma/primitive neuroectodermal tumor arising from the adrenal gland in an adolescent

Pediatric Blood and Cancer — Wed, 15 Jun 2011 23:00:00 +0100

AbstractWe review the case of an adolescent who presented with flank pain, fatigue and a discrete nonfunctioning adrenal lesion which was found to be an adrenal Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET). The patient was treated with a minimally invasive adrenalectomy as a component of multimodal therapy, including seven courses of chemotherapy and whole abdominal radiation. She is currently disease free 14 months after the operation and 3 months off therapy. Pediatr Blood Cancer © 2011 Wiley‐Liss, Inc. (Source: Pediatric Blood and Cancer)

MedWorm Sponsor Message: Please support the Doctors In Chains campaign for the medics tortured and sentenced for up to 15 years in Bahrain. #FreeDoctors

Imaging Pediatric Bone Sarcomas

Radiologic Clinics of North America — Wed, 15 Jun 2011 23:00:00 +0100

This article reviews the 3 most common pediatric bone sarcomas—osteosarcoma, Ewing sarcoma, and chondrosarcoma—and their imaging as applicable to contemporary disease staging and monitoring, and explores the roles of evolving imaging techniques. (Source: Radiologic Clinics of North America)

Superficial Ewing's sarcoma family of tumors: a clinicopathological study with differential diagnoses

Journal of Cutaneous Pathology — Wed, 08 Jun 2011 16:28:32 +0100

Conclusions: Superficial ESFT appears to have a relatively favorable prognosis but further studies with additional series, a larger number of cases and more extensive follow‐up are necessary to confirm this statement.Machado I, Llombart B, Calabuig‐Fariñas S, Llombart‐Bosch A. Superficial Ewing's sarcoma family of tumors: a clinicopathological study with differential diagnoses. (Source: Journal of Cutaneous Pathology)

A novel oncogenic mechanism in Ewing sarcoma involving IGF pathway targeting by EWS/Fli1-regulated microRNAs

Oncogene — Sun, 05 Jun 2011 23:00:00 +0100

A novel oncogenic mechanism in Ewing sarcoma involving IGF pathway targeting by EWS/Fli1-regulated microRNAs Oncogene advance online publication, June 6, 2011. doi:10.1038/onc.2011.197 Authors: E L McKinsey, J K Parrish, A E Irwin, B F Niemeyer, H B Kern, D K Birks & P Jedlicka (Source: Oncogene)

Late instrumentation failure after total en bloc spondylectomy.

Journal of Neurosurgery.Spine — Thu, 02 Jun 2011 23:00:00 +0100

Conclusions Late instrumentation failure was a frequent complication after TES. Although patients with instrumentation failure experienced back pain, the neurological sequelae were not catastrophic. For prevention, meticulous preparation of the graft site and a longer posterior fixation should be considered. PMID: 21639702 [PubMed - as supplied by publisher] (Source: Journal of Neurosurgery.Spine)

Massive venous thrombosis of inferior vena cava as primary manifestation of renal Ewing's sarcoma.

Clinical Nephrology — Tue, 31 May 2011 23:00:00 +0100

We report an extraordinarily rare case of a 17-year-old male with an extraskeletal Ewing's sarcoma (ESS) of the kidney and a massive thrombosis involving the inferior vena cava (IVC), from the iliac axis to the right atrium. This onset resembled renal cell carcinoma (RCC), although histological examination revealed it was an extraskeletal Ewing's sarcoma/peripheral neuro-ectodermal tumor (EES/PNET). EES/PNET should benefit from neoadjuvant chemotherapy to reduce the risk of metastasis and of recurrent disease due to delay in suitable treatment. Therefore, in the presence of a renal mass with tumor extension of IVC, it is reasonable to bear in mind that other tumors, apart from RCC, could occur. In such cases, a US or CT-scan guided biopsy could be useful. PMID: 21612762 [PubMed - in pr...

MedWorm Sponsor Message: Find the best January Sales in the UK.

Peripheral type of primitive neuroectodermal tumour arising from the left orbital floor.

Singapore Medical Journal — Tue, 31 May 2011 23:00:00 +0100

Authors: Santra G, Sinha PK, De D, Phaujdar S Primitive neuroectodermal tumours (PNETs) are rare tumours that originate from primitive neural crest cells. They are usually found in children below ten years of age. Peripheral PNETs (pPNETs) occur in soft tissues of the body, but have the same genetic changes as Ewing's sarcoma of the bone (now called soft tissue Ewing's sarcoma). They commonly present in the thoracopulmonary region, abdomen, pelvis and the extremities. The head and neck regions may also be involved. Our case demonstrates a PNET in the peripheral tissue arising from the left orbital floor and spreading locally to involve the left maxillofacial region, cheek and gum. The incidence of pPNETs is likely to be under-reported in the literature. Recent diagnostic advances, incl...

Combination testing (Stage 2) of the Anti‐IGF‐1 receptor antibody IMC‐A12 with rapamycin by the pediatric preclinical testing program

Pediatric Blood and Cancer — Mon, 30 May 2011 23:00:00 +0100

ConclusionsThe combination of IMC‐A12 with rapamycin was well tolerated, and induced tumor responses that were superior to either single agent alone in several models. These studies confirm reports using other antibodies that inhibit IGF‐1 receptor‐mediated signaling that indicate enhanced therapeutic effect for this combination, and extend the range of histotypes to encompass additional tumors expressing IGF‐1R where this approach may be effective. Pediatr Blood Cancer © 2011 Wiley‐Liss, Inc. (Source: Pediatric Blood and Cancer)

Treatment Outcome of Korean Patients with Localized Ewing Sarcoma Family of Tumors: A Single Institution Experience

Japanese Journal of Clinical Oncology — Thu, 26 May 2011 23:00:00 +0100

Conclusions Survival of our Ewing sarcoma family of tumors patients was not inferior to those reported for Euro-American cases. Collaborative studies are necessary for further improvements of outcome and we believe that our data provide a basis for future studies targeting Ewing sarcoma family of tumors. (Source: Japanese Journal of Clinical Oncology)

Diagnostic pitfall in the diagnosis of mesenchymal chondrosarcoma arising in the central nervous system

Neuropathology — Tue, 24 May 2011 23:00:00 +0100

We report a case of mesenchymal chondrosarcoma arising from the right tentorium cerebelli in a 21‐year‐old woman with symptoms relating to mass effect. Histological examination demonstrated a purely small round cell appearance in a specimen obtained during partial resection at an outside institution, leading to an erroneous diagnosis of Ewing sarcoma/primitive neuroectodermal tumor (PNET). The diagnosis of mesenchymal chondrosarcoma was made only after tissue obtained during a definitive complete macroscopic removal involving the regional tentorium cerebelli, transverse and sigmoid dural venous sinuses which showed a prominent cartilaginous component. We discuss the features of mesenchymal chondrosarcoma arising in the CNS, the important differential diagnoses of small round‐cell tum...

Case: Delay in diagnosis and treatment of Ewing's sarcoma: Master Osman Mcauley Gocmen (by his mother and litigation friend Mrs Shannie Gocmen)-v-University Hospitals Bristol NHS foundation trust

Clinical Risk — Mon, 23 May 2011 23:00:00 +0100

(Source: Clinical Risk)

MedWorm Sponsor Message: Please support the Doctors In Chains campaign for the medics tortured and sentenced for up to 15 years in Bahrain. #FreeDoctors

Analysis of Prognostic Factors of Pediatric-Type Sarcomas in Adult Patients.

Oncology — Sun, 22 May 2011 23:00:00 +0100

Conclusion: We identified the prognostic variables which may facilitate risk-adapted therapies for this rare adult sarcoma group, which should be further investigated. PMID: 21606660 [PubMed - as supplied by publisher] (Source: Oncology)

Immunohistochemical Staining for TLE1 Distinguishes Synovial Sarcoma From Histologic Mimics.

American Journal of Clinical Pathology — Wed, 18 May 2011 20:01:29 +0100

In this study, only a small subset of MPNSTs and SFTs showed limited staining for TLE1. PMID: 21571956 [PubMed - in process] (Source: American Journal of Clinical Pathology)

Mechanistic Rationale for Inhibition of Poly(ADP-Ribose) Polymerase in ETS Gene Fusion-Positive Prostate Cancer

Cancer Cell — Tue, 17 May 2011 00:00:00 +0100

J. Chad Brenner, Bushra Ateeq, Yong Li, Anastasia K. Yocum, Qi Cao, Irfan A. Asangani, Sonam Patel, Xiaoju Wang, Hallie Liang, Jindan Yu, Nallasivam Palanisamy, Javed Siddiqui, Wei Yan, Xuhong Cao, Rohit Mehra, Aaron Sabolch, Venkatesha Basrur, Robert J. Lonigro, Jun Yang, Scott A. Tomlins, Christopher A. Maher, Kojo S.J. Elenitoba-Johnson, Maha Hussain, Nora M. Navone, Kenneth J. Pienta, Sooryanarayana Varambally, Felix Y. Feng, Arul M. Chinnaiyan. Recurrent fusions of ETS genes are considered driving mutations in a diverse array of cancers, including Ewing's sarcoma, acute myeloid leukemia, and prostate cancer. We investigate the mechanisms.... (Source: Cancer Cell)

Four year experience of sarcoma of soft tissues and bones in a tertiary care hospital and review of literature

World Journal of Surgical Oncology — Mon, 16 May 2011 23:00:00 +0100

Conclusion: The median recurrence free survival of patients in our study was comparable to the reported literature but with significant lost to follow rate. Further large-scale, multi centre studies are needed to have a more comprehensive understanding of this heterogeneous disease in our population. (Source: World Journal of Surgical Oncology)

Initial testing of the CENP‐E inhibitor GSK923295A by the pediatric preclinical testing program

Pediatric Blood and Cancer — Sun, 15 May 2011 23:00:00 +0100

ConclusionsGSK923295A demonstrated significant antitumor activity against solid tumor models, inducing CRs in Ewing sarcoma, rhabdoid, and rhabdomyosarcoma xenografts. These results suggest that CENP‐E may be a valuable therapeutic target in pediatric cancer. Pediatr Blood Cancer © 2011 Wiley‐Liss, Inc. (Source: Pediatric Blood and Cancer)

MedWorm Sponsor Message: Find the best January Sales in the UK.

Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney: Report of a case diagnosed by fine needle aspiration cytology and confirmed by immunocytochemistry and RT‐PCR along with review of literature

Diagnostic Cytopathology — Tue, 03 May 2011 23:00:00 +0100

We report a rare case of ES/PNET of the kidney in a 35‐year‐old female patient diagnosed by fine needle aspiration cytology (FNAC) and confirmed by immunohistochemistry (IHC) and reverse‐transcriptase polymerase chain reaction (RT‐PCR). Ultrasound guided FNAC smears from the kidney mass showed a population of malignant small round cells with perivascular arrangement and focal rosette formation. IHC performed on the cell block, showed strong immunopositivity for CD99 (MIC2) and vimentin. Molecular analysis of the aspirate by RT‐PCR confirmed the EWS‐FLI type1 transcript. The application of RT‐PCR on FNAC material for establishing a diagnosis of renal ES/PNET is being reported for the first time. FNAC also confirmed metastases in the right level I cervical lymph node. The utili...

Investigational Agent Shows Promise In Reducing Spread Of Prostate Cancer

Health News from Medical News Today — Sun, 01 May 2011 08:00:00 +0100

A drug developed to treat Ewing's Sarcoma, a rare childhood cancer, may also help prevent human prostate cancer from spreading, as seen in new lab studies say researchers at Georgetown Lombardi Comprehensive Cancer Center, a part of Georgetown University Medical Center (GUMC). Published online April 29 in PLoS ONE, the researchers report that if the agent continues to work well in further laboratory and preclinical studies, it may be the first prostate cancer drug specifically designed to stop cancer spread, or metastasis... (Source: Health News from Medical News Today)

Distal femoral physeal growth arrest secondary to a cemented proximal femoral endoprosthetic replacement.

The Journal of Bone and Joint Surgery. British volume — Sat, 30 Apr 2011 23:00:00 +0100

We report a case of spontaneous physeal growth arrest of the distal femur in a nine-year-old child with Ewing's sarcoma of the proximal femur treated with chemotherapy and endoprosthetic replacement. Owing to the extent of disuse osteoporosis at the time of surgery, the entire intramedullary canal up to the distal femoral physis was filled with cement. Three years later, the femur remained at its pre-operative length of 19 cm. Pre-operative calculations of further growth failed to account for the growth arrest, and the initial expandable growing prosthesis inserted has been revised to a longer one in order to address the leg-length discrepancy. To our knowledge, this is the only reported case of distal femoral physeal growth arrest following cemented endoprosthetic replacement of the proxi...

Lung nodules in pediatric oncology patients: a prediction rule for when to biopsy

Journal of Pediatric Surgery — Sat, 30 Apr 2011 23:00:00 +0100

Conclusions: Lesions that are between 5 and 10 mm in size and peripherally located in patients with osteosarcoma, Ewing sarcoma, or hepatocellular carcinoma are most likely to be malignant. Use of a prediction rule can help guide clinical practice by determining which patients should undergo surgical resection of lung nodules and which patients may be closely observed with continued radiologic studies. (Source: Journal of Pediatric Surgery)

Targeting the insulin growth factor pathway in gastrointestinal cancers.

Oncology (Williston Park, N.Y.) — Sat, 30 Apr 2011 23:00:00 +0100

Authors: Golan T, Javle M Insulin-like growth factor 1 (IGF-1)-directed therapy is currently at a crossroads. After decades of research, several agents targeting the IGF pathway are now in clinical trials. One recent phase III trial of the IGF-1R inhibitor figitumumab in patients with non-small-cell lung cancer was discontinued after an interim analysis showed no survival improvement. Clinical trials for patients with sarcoma have demonstrated impressive anti-tumor activity in cases where the IGF-1 pathway is activated, such as in Ewing sarcoma; however, acquired resistance has been common. Recently, randomized phase II trials combining IGF-1 R with epidermal grown factor receptor (EGFR) inhibition in colorectal cancer have been completed. Preclinical studies have indicated that severa...

MedWorm Sponsor Message: Please support the Doctors In Chains campaign for the medics tortured and sentenced for up to 15 years in Bahrain. #FreeDoctors

Investigational agent shows promise in reducing spread of prostate cancer

ScienceDaily Headlines — Sat, 30 Apr 2011 00:22:22 +0100

A drug developed to treat Ewing's Sarcoma, a rare childhood cancer, may also help prevent human prostate cancer from spreading, as seen in new lab studies. (Source: ScienceDaily Headlines)

Nonmetastatic Ewing family tumors: high-dose chemotherapy with stem cell rescue in poor responder patients. Results of the Italian Sarcoma Group/Scandinavian Sarcoma Group III protocol

Annals of Oncology — Mon, 25 Apr 2011 23:00:00 +0100

Conclusions: High-dose therapy added to the VACA-IE regimen in PR patients is feasible and effective. Selected groups of patients with ES can benefit from HDT. (Source: Annals of Oncology)

Intra‐abdomen Ewing's sarcoma

ANZ Journal of Surgery — Mon, 25 Apr 2011 05:46:34 +0100

(Source: ANZ Journal of Surgery)

Orbital Infantile Myofibroma: a Case Report and Clinicopathologic Review of 24 Cases from the Literature

Head and Neck Pathology — Thu, 21 Apr 2011 18:01:26 +0100

Abstract Isolated orbital infantile myofibroma are rare tumors in the head and neck. The mass-like clinical presentation and variable histologic features result in frequent misdiagnosis and potentially inappropriate clinical management. There are only a few reported cases in the English literature. Twenty-four patients with orbital infantile myofibroma or myofibromatosis were compiled from the English literature (Medline 1960–2011) and integrated with this case report. The patients included 14 males and 10 females, aged newborn to 10 years (mean, 34.8 months), who presented with a painless mass in the infra- or supraorbital regions, usually increasing in size andassociated with exophthalmos (n = 5). Females were on average older than their male counter...

MedWorm Sponsor Message: Find the best January Sales in the UK.

Dose escalation of intravenous irinotecan using oral cefpodoxime: A phase I study in pediatric patients with refractory solid tumors

Pediatric Blood and Cancer — Tue, 19 Apr 2011 23:00:00 +0100

ConclusionsThe MTD of intravenous irinotecan administered on a protracted schedule was increased by 50% from 20 to 30 mg/m2/dose with the addition of oral cefpodoxime. The most prominent DLT remained diarrhea. High interpatient variability in irinotecan pharmacokinetics was observed; however, SN‐38 exposure at the MTD was greater than most reported exposures with the 20 mg/m2 dosage. Pediatr Blood Cancer © 2011 Wiley‐Liss, Inc. (Source: Pediatric Blood and Cancer)

Therapy-Related Acute Myeloid Leukemia following Treatment with Trabectedin for Ewing's Sarcoma.

Acta Haematologica — Sun, 17 Apr 2011 23:00:00 +0100

Authors: Liu SV, Zneimer S, Tahbaz A, Douer D No abstract available. PMID: 21502754 [PubMed - as supplied by publisher] (Source: Acta Haematologica)

Investigation of the insulin‐like growth factor‐1 signaling pathway in localized Ewing sarcoma

Cancer — Thu, 07 Apr 2011 23:00:00 +0100

CONCLUSIONS:The baseline IGFBP‐3:IGF‐1 ratio was correlated with age but did not affect the outcomes of patients with ES. The authors concluded that additional investigation of the IGF‐1 pathway is warranted in patients with ES, and especially in those who have received treatment with IGF‐1 receptor antibody inhibitors. Cancer 2011;. © 2011 American Cancer Society. (Source: Cancer)

EUS-FNA for the Diagnosis of Retroperitoneal Primitive Neuroectodermal Tumor

Comparative and Functional Genomics — Thu, 07 Apr 2011 14:08:53 +0100

Primitive neuroectodermal tumor (PNET) is a rare “small round blue cell tumor” that is diagnosed by open biopsy or percutaneous biopsy of the lesion under radiologic guidance. In this case report, we present a novel approach to the diagnosis of a retroperitoneal PNET by endoscopic ultrasound- (EUS-) guided fine needle aspiration (FNA). A 35-year-old man presented with the history of left-sided flank pain and swelling of 3-weeks duration. Computerized tomography (CT) scan of his abdomen revealed a 12.8 × 13 × 12.5 cm cystic and solid mass arising from the retroperitoneum and displacing the third and fourth portions of the duodenum. He underwent EUS which revealed a well-circumscribed heterogeneous mass abutting the inferior portion of the stomach. EUS-FNA of...

Excellent prognosis in a subset of patients with Ewing sarcoma identified at diagnosis by CD56 using flow cytometry.

Clinical Cancer Research — Mon, 04 Apr 2011 23:00:00 +0100

CONCLUSIONS: All samples contained cells that are positive for the CD99+/CD90+/CD45-combination at diagnosis indicating that ES is a systemic disease. CD56 expression could be used to reveal ES patients predisposed to relapse, thus improving treatment stratification and implementation of personalized therapy. PMID: 21467162 [PubMed - as supplied by publisher] (Source: Clinical Cancer Research)

MedWorm Sponsor Message: Please support the Doctors In Chains campaign for the medics tortured and sentenced for up to 15 years in Bahrain. #FreeDoctors

Application of flow cytometry in pediatric hematology‐oncology

Pediatric Blood and Cancer — Thu, 31 Mar 2011 23:00:00 +0100

AbstractApplications of flow cytometry in pediatric cancers have expanded substantially in recent years. In acute leukemias, the commonest childhood cancer, flow cytometry can now define complex antigenic profiles that are associated with specific cytogenetic/molecular defects and can also directly identify BCR‐ABL fusion protein. Flow cytometry based scoring system has been described for diagnosis of myelodysplastic syndromes. In solid tumors, flow cytometry was previously used mainly to determine DNA content for prognosis; however, recent studies in children with neuroblastoma and Ewing sarcoma have identified its diagnostic utility. In this review, we will discuss the current and future applications of flow cytometry in pediatric hematology‐oncology. Pediatr Blood Cancer © 2011 Wil...

Oncological outcomes of patients with Ewing's sarcoma: IS THERE A DIFFERENCE BETWEEN SKELETAL AND EXTRA-SKELETAL EWING'S SARCOMA?

The Journal of Bone and Joint Surgery. British volume — Thu, 31 Mar 2011 23:00:00 +0100

Authors: Pradhan A, Grimer RJ, Spooner D, Peake D, Carter SR, Tillman RM, Abudu A, Jeys L The aim of this study was to identify whether there was any difference in patient, tumour, treatment or outcome characteristics between patients with skeletal or extra-skeletal Ewing's sarcoma. We identified 300 patients with new primary Ewing's sarcoma diagnosed between 1980 and 2005 from the centres' local database. There were 253 (84%) with skeletal and 47 (16%) with extra-skeletal Ewing's sarcomas. Although patients with skeletal Ewing's were younger (mean age 16.8 years) than those with extra-skeletal Ewing's sarcoma (mean age 27.5 years), there was little difference between the groups in terms of tumour stage or treatment. Nearly all the patients were treated with chemotherapy and most had s...

Chromosome banding analysis of cells from fine-needle aspiration biopsy samples from soft tissue and bone tumors: is it clinically meaningful?

Cancer Genetics and Cytogenetics — Thu, 31 Mar 2011 23:00:00 +0100

Morphologic evaluation of samples from fine-needle aspiration (FNA) and core needle (CN) biopsies is an important part of the pretreatment diagnosis of bone and soft tissue tumors. Because most such tumors have characteristic, sometimes even specific, chromosomal rearrangements, ancillary genetic analyses could provide important diagnostic information. Whereas directed analyses, such as fluorescence in situ hybridization or reverse transcriptase–polymerase chain reaction, for specific genetic aberrations are well suited for the relatively small cell numbers obtained with FNA biopsies, the possibility to obtain tumor karyotypes after cell culturing has been less well studied. In the present study, karyotypes from 114 FNA biopsy samples were compared to those in corresponding surgical tumo...

Efficacy and pharmacokinetic/pharmacodynamic evaluation of the Aurora kinase A inhibitor MLN8237 against preclinical models of pediatric cancer

Cancer Chemotherapy and Pharmacology — Tue, 29 Mar 2011 17:32:30 +0100

Conclusions Objective responses were more frequent in tumors with decreased AURKA copy number (5/8) compared to those with increased gene copy number (2/14). This report confirms the significant activity against both solid tumor and ALL xenografts at the MTD, with a steep dose response. These data support clinical development of MLN8237 in childhood cancer. Because of the steep dose–response relationship, such studies should target achieving trough levels of 1 μM or higher for sustained periods of treatment. Content Type Journal ArticlePages 1-14DOI 10.1007/s00280-011-1618-8Authors Hernan Carol, Leukaemia Biology Program, Children’s Cancer Institute Australia for Medical Research, Lowy Cancer Research Centre, UNSW, Randwick, NSW 2031, AustraliaIngrid Boeh...

Clinical outcomes of rhabdomyosarcoma and Ewing's sarcoma of the head and neck in children

Auris, Nasus, Larynx — Tue, 29 Mar 2011 16:34:56 +0100

Conclusion: Treatment for rhabdomyosarcoma and Ewing's sarcoma consists in a multimodal therapy involving chemotherapy, radiotherapy and surgery. The optimum use, timing and intensity of these three treatments are still matters of international debate. Chemotherapy in association with radiotherapy has proven capable to obtain local and distant control of disease. But when surgery is unfeasible or fails in radicality, local control is difficult without radiotherapy. Despite additional therapeutic efforts, prognosis of relapsing disease remains poor. (Source: Auris, Nasus, Larynx)

MedWorm Sponsor Message: Find the best January Sales in the UK.

Risk of recurrence and survival after relapse in patients with Ewing sarcoma

Pediatric Blood and Cancer — Fri, 25 Mar 2011 15:23:44 +0100

Conclusions5‐year OSr in Ewing sarcoma is poor (<0.2). Prognostically favorable factors are: late onset (>2 years) and strictly localized relapse. Pediatr Blood Cancer © 2011 Wiley‐Liss, Inc. (Source: Pediatric Blood and Cancer)

Local control of metastatic sites with radiation therapy in metastatic Ewing sarcoma and rhabdomyosarcoma

Pediatric Blood and Cancer — Thu, 24 Mar 2011 00:00:00 +0100

AbstractApproximately 20% of children with Ewing sarcoma (EWS) and rhabdomyosarcoma (RMS) present with metastatic disease at initial diagnosis. Overall, the outcome is poor, with an event‐free survival of <20%. Local control at metastatic sites has not been previously reported. We reviewed control of metastatic sites in children with EWS and RMS that received curative intent radiation therapy to each metastatic site. In 13 children, at a median follow‐up of 18 months, a single local failure was seen. Toxicity was minimal. Our data suggest that radiation therapy is effective and tolerable in children with metastatic EWS and RMS. Pediatr Blood Cancer © 2011 Wiley‐Liss, Inc. (Source: Pediatric Blood and Cancer)

Second neoplasm in children treated in EORTC 58881 trial for acute lymphoblastic malignancies: Low incidence of CNS tumours

Pediatric Blood and Cancer — Tue, 15 Mar 2011 00:00:00 +0100

ConclusionThe overall incidence rate of SN is comparable to that reported previously. In spite of short follow‐up time, the low incidence of brain tumours might be related to the omission of cranial radiotherapy. Pediatr Blood Cancer © 2011 Wiley‐Liss, Inc. (Source: Pediatric Blood and Cancer)

Treatment of Ewing sarcoma family of tumors with a modified P6 protocol in children and adolescents

Pediatric Blood and Cancer — Mon, 07 Mar 2011 00:00:00 +0100

ConclusionsIn this pilot study, the mP6 protocol produced a complete remission rate of 83% at 4 years in non‐metastatic ESFT reducing the risk of secondary malignancies. Pediatr Blood Cancer © 2011 Wiley‐Liss, Inc. (Source: Pediatric Blood and Cancer)

Auto-stimulatory action of secreted caveolin-1 on the proliferation of Ewing's sarcoma cells.

International Journal of Oncology — Thu, 03 Mar 2011 00:00:00 +0100

Authors: Sengupta A, Mateo-Lozano S, Tirado OM, Notario V Caveolin-1 (CAV1) is highly expressed in Ewing's sarcoma (EWS). We previously showed that increased cellular CAV1 is associated with the regulation of the tumorigenicity, drug resistance and metastatic ability of EWS cells. Because several studies reported that melanoma and prostate cancer cells, which express relatively high CAV1 levels, secrete CAV1, and that secreted CAV1 is associated with tumor progression, our study explored the possibility that EWS cells also secreted CAV1 and that secreted CAV1 may contribute to EWS pathobiology. Results from experiments involving the ectopic expression of a Myc-tagged CAV1 protein in EWS cells as well as the supplementation of culture media with purified CAV1 protein followed by its int...

MedWorm Sponsor Message: Please support the Doctors In Chains campaign for the medics tortured and sentenced for up to 15 years in Bahrain. #FreeDoctors

Initial testing (stage 1) of the polyamine analog PG11047 by the pediatric preclinical testing program

Pediatric Blood and Cancer — Sat, 26 Feb 2011 15:20:21 +0100

ConclusionsFurther pediatric development of PG11047 will require better defining a target population and identifying combinations for which there is a tumor‐selective cytotoxic effect. The regression observed for an ependymoma xenograft and the exquisite sensitivity of some Ewing sarcoma cell lines to the antiproliferative effects of PG11047 provide leads for further preclinical investigations. Pediatr Blood Cancer © 2011 Wiley‐Liss, Inc. (Source: Pediatric Blood and Cancer)

Regulation of the expression of death receptors and their ligands by melatonin in haematological cancer cell lines and in leukaemia cells from patients

Journal of Pineal Research — Thu, 24 Feb 2011 00:00:00 +0100

Abstract: Incorporation of new therapeutic agents remains as a major challenge for treatment of patients with malignant haematological disorders. Melatonin is an indolamine without relevant side effects. It has been shown previously to exhibit synergism with several chemotherapeutic drugs in Ewing sarcoma cells by potentiating the extrinsic pathway of apoptosis. It also sensitizes human glioma cells against TRAIL by increasing DR5 expression. Here, we report the induction of cell death by melatonin in several human malignant haematological cell lines through the activation of the extrinsic pathway of apoptosis. Such activation was mediated by the increase in the expression of the death receptors Fas, DR4 and DR5 and their ligands Fas L and TRAIL, with a remarkable rise in the expression...

Ectopic ACTH Syndrome in Children and Adolescents.

The Journal of Clinical Endocrinology and Metabolism — Wed, 23 Feb 2011 00:00:00 +0100

Conclusions: EAS in youngsters displays many similarities to that described in adults.The diagnostic and therapeutic algorithms recommended in adults can be used in this population. PMID: 21346064 [PubMed - as supplied by publisher] (Source: The Journal of Clinical Endocrinology and Metabolism)

Relationship between height at diagnosis and bone tumours in young people: a meta-analysis

Cancer Causes and Control — Fri, 18 Feb 2011 17:28:26 +0100

Conclusion The average height of patients with osteosarcoma, but not Ewing sarcoma, was significantly above the average height of the reference population by 2–3 centimetres. The observed differences indicate the involvement of pubertal longitudinal bone growth in osteosarcoma development while different biological pathways could be relevant for Ewing sarcoma. Content Type Journal ArticlePages 1-8DOI 10.1007/s10552-011-9740-9Authors Ramandeep S. Arora, Cancer Research UK Paediatric and Familial Cancer Research Group, 1.906, University of Manchester, Stopford Building, Oxford Road, M13 9PL Manchester, UKEvangelos Kontopantelis, Health Sciences Primary Care Research Group, University of Manchester, Manchester, UKRobert D. Alston, Cancer Research UK Paediatric and Fa...

18F-FDG PET response to neoadjuvant chemotherapy for Ewing sarcoma and osteosarcoma are different

Skeletal Radiology — Sun, 06 Feb 2011 06:38:55 +0100

Conclusion Response to neoadjuvant chemotherapy as reflected by changes in PET characteristics should be interpreted differently for ES and OS. Content Type Journal ArticlePages 1-9DOI 10.1007/s00256-011-1096-4Authors Louie L. Gaston, Department of Orthopedics, Philippine General Hospital, Manila, PhilippinesClaudia Di Bella, Department of Orthopedics, Saint Vincent’s Hospital, Lev 3, Daly Wing, 41 Victoria Pde, East Melbourne, VIC 3065, AustraliaJohn Slavin, Department of Pathology, Saint Vincent’s Hospital, Melbourne, AustraliaRodney J. Hicks, Centre for Cancer Imaging, Translational Research Group, Molecular Imaging and Targeted Therapeutics Laboratory, The Peter MacCallum Cancer Centre, East Melbourne, VIC AustraliaPeter F. M. Choong, Department of Orthopedic...

MedWorm Sponsor Message: Find the best January Sales in the UK.

Ewing sarcoma cells express rankl and support osteoclastogenesis

The Journal of Pathology — Thu, 03 Feb 2011 00:00:00 +0100

AbstractEwing sarcoma (ES) is a primary malignant round cell tumour of bone characterised by rapid and extensive osteolysis. Cellular mechanisms underlying the rapid bone resorption in ES have not been characterised. Osteoclasts are marrow‐derived multinucleated cells that effect tumour osteolysis. The role of ES tumour cells in influencing osteoclast formation and/or directly contributing to the osteolysis in ES has not been determined. Using a tissue culture bioassay we found that lacunar resorption is not carried out by (CD99+) ES tumour cells, but by (CD68+) macrophage/osteoclast‐like cells; this resorption occurred in the absence of the osteoclastogenic factor, receptor activator for nuclear factor B ligand (RANKL). ES cell lines cultured directly on dentine slices did not resorb ...

FUS/TLS Is a Novel Mediator of Androgen-Dependent Cell-Cycle Progression and Prostate Cancer Growth

Cancer Research — Tue, 01 Feb 2011 00:00:00 +0100

Progression of prostate cancer is highly dependent upon the androgen receptor pathway, such that knowledge of androgen-regulated proteins is vital to understand and combat this disease. Using a proteomic screen, we found the RNA-binding protein FUS/TLS (Fused in Ewing's Sarcoma/Translocated in Liposarcoma) to be downregulated in response to androgen. FUS has recently been shown to be recruited by noncoding RNAs to the regulatory regions of target genes such as cyclin D1, in which it represses transcription by disrupting complex formation. Here we show that FUS has some characteristics of a putative tumor suppressor, as its overexpression promoted growth inhibition and apoptosis of prostate cancer cells, whereas its knockdown increased cell proliferation. This effect was reproducible in viv...

Primary peripheral PNET/Ewing's sarcoma arising in the meninges, confirmed by the presence of the rare translocation t(21;22) (q22;q12)

Neuropathology — Tue, 01 Feb 2011 00:00:00 +0100

We report a case of 37‐year‐old man with pPNET/ES arising in the meninges and bearing the rare translocation t(21;22) (q22;q12). The tumor was composed of sheets and nests of monotonous small cells with round to oval nuclei, finely dispersed chromatin, small nucleolus and scant cytoplasm. We discuss the importance of the differential diagnosis with central primitive neuroectodermal tumors (cPNET). (Source: Neuropathology)

Resection of pulmonary metastases in pediatric patients with Ewing sarcoma improves survival

Journal of Pediatric Surgery — Tue, 01 Feb 2011 00:00:00 +0100

Conclusion: These data suggest a possible benefit for ES patients who undergo surgical resection of lung metastases. (Source: Journal of Pediatric Surgery)

Ecteinascidin 743 Interferes with the Activity of EWS-FLI1 in Ewing Sarcoma Cells.

Neoplasia — Tue, 01 Feb 2011 00:00:00 +0100

In this report, we show that modulation of specific oncogenic transcription factors by ET-743 may extend to other tumor types. We demonstrate that, among a panel of pediatric sarcomas, Ewing sarcoma family of tumors (ESFTs) cell lines bearing the EWS-FLI1 transcription factor are the most sensitive to treatment with ET-743 compared with osteosarcoma, rhabdomyosarcoma, and synovial sarcoma. We show that ET-743 reverses a gene signature of induced downstream targets of EWS-FLI1 in two different ESFT cell lines (P = .001). In addition, ET-743 directly suppresses the promoter activity of a known EWS-FLI1 downstream target NR0B1 luciferase reporter construct without changing the activity of a constitutively active control in ESFT cells. Furthermore, the effect is specific to EWS-FLI1, as forced...

MedWorm Sponsor Message: Please support the Doctors In Chains campaign for the medics tortured and sentenced for up to 15 years in Bahrain. #FreeDoctors

Primary malignant bone tumors—recent developments

Seminars in Diagnostic Pathology — Tue, 01 Feb 2011 00:00:00 +0100

Primary malignant bone tumors are rare sarcomas with an estimated frequency of about 2900 new cases per year; they constitute less than 0.2% of all cancers diagnosed in the United States. The diagnosis and management of these neoplasms require a team approach, which includes orthopaedic surgeons, radiologists, pathologists, and oncologists. With this approach and current treatment modalities, the 5-year survival for the most common malignant bone tumors, osteosarcoma and Ewing sarcoma, are 70% and 60%, respectively. This review will summarize recent developments and advances in molecular pathogenesis of the more common primary malignant bone neoplasms. (Source: Seminars in Diagnostic Pathology)

Efficacy of and resistance to anti-IGF-1R therapies in Ewing's sarcoma is dependent on insulin receptor signaling

Oncogene — Mon, 31 Jan 2011 00:00:00 +0100

Authors: C Garofalo, M C Manara, G Nicoletti, M T Marino, P-L Lollini, A Astolfi, G Pandini, J A López-Guerrero, K-L Schaefer, A Belfiore, P Picci & K Scotlandi (Source: Oncogene)

Ewing sarcoma gene Ews regulates hematopoietic stem cell senescence

Blood — Thu, 27 Jan 2011 00:00:00 +0100

The longevity of organisms is maintained by stem cells. If an organism loses the ability to maintain a balance between quiescence and differentiation in the stem/progenitor cell compartment due to aging and/or stress, this may result in death or age-associated diseases, including cancer. Ewing sarcoma is the most lethal bone tumor in young patients and arises from primitive stem cells. Here, we demonstrated that endogenous Ewing sarcoma gene (Ews) is indispensable for stem cell quiescence, and that the ablation of Ews promotes the early onset of senescence in hematopoietic stem progenitor cells. The phenotypic and functional changes in Ews-deficient stem cells were accompanied by an increase in senescence-associated β-galactosidase staining and a marked induction of p16INK4a compared ...

The Evaluation of CD99 Immunoreactivity and EWS/FLI1 Translocation by Fluorescence in situ Hybridization in Central PNETs and Ewing's Sarcoma Family of Tumors.

Pathology Oncology Research — Wed, 26 Jan 2011 00:00:00 +0100

In conclusion, CD99 expression and EWS/FLI1 translocation are specific and sensitive markers in the diagnosis of ESFTs. However, these were often not found in cases of cPNET. Therefore, in the diagnosis of ESFTs, clinical, radiological, histopathological and immunohistochemical parameters should always be evaluated together. PMID: 21267687 [PubMed - as supplied by publisher] (Source: Pathology Oncology Research)