MedWorm: Desmoplastic Small Round Cell Tumor (DSRCT)

Updates on the multimodality management of desmoplastic small round cell tumor

Journal of Surgical Oncology — Tue, 03 Jan 2012 05:00:00 +0100

AbstractDesmoplastic Small Round Cell Tumor (DSRCT) is a rare and an aggressive malignancy with poor outcome. This tumor can co‐express epithelial, neural, and mesenchymal markers. The molecular hallmark of DSRCT is the EWS–WT1 fusion protein. Despite the diversities in treatment modality, the best results have been seen with radical surgery and adjuvant or neoadjuvant chemotherapy. J. Surg. Oncol © 2011 Wiley Periodicals, Inc. (Source: Journal of Surgical Oncology)

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Desmoplastic small round cell tumors with EWS‐WT1 fusion transcript in children and young adults

Pediatric Blood and Cancer — Sun, 11 Dec 2011 05:00:00 +0100

ConclusionsDetection of the EWS‐WT1‐GFT plays a major role in the diagnosis of DSRCT. No survival difference was observed according to extent of surgery, but complete surgery seemed to offer the best chance of long‐term survival. High‐dose chemotherapy or local radiotherapy did not appear to improve survival in this retrospective analysis, but larger prospective studies are needed to provide definitive conclusions on the role of these treatments. 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)

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...

Utility of ultrasound‐guided fine‐needle aspiration in splenic lesions

Diagnostic Cytopathology — Mon, 26 Sep 2011 04:00:00 +0100

AbstractIndications of fine‐needle aspiration (FNA) of spleen have increased as more splenic lesions are detected because of advanced imaging techniques. A retrospective analysis of cytological material of 36 patients on whom ultrasound‐guided splenic FNA was performed was done. No complications were noted. There were 16 inflammatory lesions, 12 neoplastic and 8 cases were reported as descriptive either because of scant cellularity, blood only, or normal splenic cytology. Inflammatory lesions included nine cases of acute abscess, five cases of tuberculosis, and one case each of leishmaniasis and infarct. Neoplastic lesions included two benign (benign cyst and inflammatory pseudotumor) and 10 malignant lesions. Among malignant lesions, eight were non‐Hodgkin lymphoma (NHL), one suspic...

An Unusual Presentation of Desmoplastic Small Round Cell Tumour of the Abdomen: Morphological, Immunohistochemical, Ultrastructural, and Molecular Studies

Advances in Urology — Mon, 19 Sep 2011 15:19:21 +0100

We report on a 34-year-old male who had abdominal discomfort with a large intraperitoneal mass. Histological examination of the tumour biopsy revealed sheets of small round cells. The cells were positive with vimentin and desmin (with occasional dot positivity) and negative for WT1 and CD 99 with immunohistochemistry. Cytogenetics showed a translocation disrupting the EWSR 1 gene on 22 q 12 consistent with DSRCT. Electron microscopic examination showed sparse cytoplasmic organelles. The patient succumbed 34 months from disease presentation after multiple chemotherapies and thereafter radiotherapy. In summary, our case exemplifies that it is crucial to combine clinical, histological, and molecular aspects in diagnosing DSRCT especially when characteristic dot positivity with desmin is weak ...

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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)

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)

Desmoplastic small round cell tumour in a 74 year old man: an uncommon cause of ascites (case report)

Diagnostic Pathology — Wed, 22 Jun 2011 23:00:00 +0100

A rare case is provided of a 74 year old man who presented with ascites of unknown etiology. CT scan of the abdomen revealed extensive omental caking, and omental biopsy cytogenetics showed findings in keeping with a diagnosis of desmoplastic small round cell tumour (DSRCT). This case is unique in that it involves a significantly older patient, negative WT1 immunohistochemical staining, and negative cytology. Despite repeated paracenteses and fluid management, the patient died in hospital secondary to renal complications. (Source: Diagnostic Pathology)

Lysine-specific demethylase 1 is highly expressed in solitary fibrous tumors, synovial sarcomas, rhabdomyosarcomas, desmoplastic small round cell tumors, and malignant peripheral nerve sheath tumors

Human Pathology — Mon, 02 May 2011 04:00:00 +0100

Summary: Epigenetic changes including histone methylation, histone acetylation, and DNA methylation are thought to play important roles in the onset and progression of cancer in numerous tumor types. Recent evidence shows that dysregulated epigenetic modifications are as significant as genetic mutations and can act as oncogenic driver lesions causing autonomous growth of cancer cells. Here, we investigated the role of lysine-specific demethylase 1 in mesenchymal tumors. Lysine-specific demethylase 1 is the first discovered histone lysine demethylase and can demethylate both H3K4me2/1 and H3K9me2/1. By analyzing a total of 468 tumors, we describe for the first time high lysine-specific demethylase 1 expression in several highly malignant sarcomas, including synovial sarcomas, rhabdomyosarco...

[90Y]Yttrium Microspheres Radioembolotherapy in Desmoplastic Small Round Cell Tumor Hepatic Metastases [DIAGNOSIS IN ONCOLOGY]

Journal of Clinical Oncology — Wed, 06 Apr 2011 23:00:00 +0100

(Source: Journal of Clinical Oncology)

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Desmoplastic small round cell tumor of the pancreas: An unusual primary site for an uncommon tumor

Journal of Indian Association of Pediatric Surgeons — Fri, 18 Mar 2011 00:00:00 +0100

Sajid S Qureshi, Shailesh Shrikhande, Mukta Ramadwar, Saral Desai, Seethalakhsmi Visvanathan, Seema S Medhi, Siddharth Laskar, Mary A Muckaden, Suresh K Pai, Sangeeta Desai, Purna A KurkureJournal of Indian Association of Pediatric Surgeons 2011 16(2):66-68Pancreas is a rare location for desmoplastic small round cell tumor. The present case highlights the dilemma in diagnosis and ascertaining the site of tumor origin. Morphologic and immunohistochemical features were complemented with the molecular markers and tumor origin which was initially nebulous was subsequently confirmed on exploratory laparotomy. (Source: Journal of Indian Association of Pediatric Surgeons)

Desmoplastic Small Round Cell Tumor of the Lung [CASE REPORTS]

The Annals of Thoracic Surgery — Wed, 01 Dec 2010 00:00:00 +0100

We report a rare case of desmoplastic small round cell tumor, which arose from the left lung. A 25-year-old man was found to have an abnormal shadow during a routine physical examination and was admitted to our hospital. A thoracoscopic tumor biopsy was performed under general anesthesia. According to the histopathologic findings of permanent sections, the tumor was composed of sharply outlined nests, clusters, and trabeculae of small round to oval cells separated by a spindle-shaped desmoplastic stroma. A gene analysis revealed chimera genes of Ewing's sarcoma and Wilms' tumor by reverse transcription polymerase chain reaction. (Source: The Annals of Thoracic Surgery)

Utilization of fluorescence in situ hybridization in the diagnosis of 230 mesenchymal neoplasms: an institutional experience.

Archives of Pathology and Laboratory Medicine — Wed, 01 Dec 2010 00:00:00 +0100

Conclusion-FISH is a useful adjunct in the diagnosis of mesenchymal neoplasms. PMID: 21128778 [PubMed - in process] (Source: Archives of Pathology and Laboratory Medicine)

Desmoplastic small round cell tumor of the lung.

The Annals of Thoracic Surgery — Sat, 27 Nov 2010 05:47:52 +0100

Recent advances in the molecular diagnosis of paediatric soft tissue sarcomas

Diagnostic Histopathology — Mon, 08 Nov 2010 00:00:00 +0100

Abstract: Sarcomas comprise a significant portion of solid tumours diagnosed in children. As they commonly are composed of primitive round or spindled cells, morphologic diagnosis can be difficult, and ancillary studies are often necessary. In recent years, progress has been made in understanding the cytogenetic and molecular changes underlying many of these tumours, and molecular techniques have emerged from strictly being research tools to becoming standard diagnostic tests. Paediatric sarcomas fall into two categories: those with characteristic underlying translocations, and those without. Translocation associated paediatric sarcomas include, but are not limited to alveolar rhabdomyosarcoma, Ewing’s family of tumours, desmoplastic small round cell sarcoma, synovial sarcoma, infantile ...

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Activity of trabectidin in desmoplastic small round cell tumor

Medical Oncology — Wed, 06 Oct 2010 06:04:42 +0100

Abstract Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive cancer that typically arises in teenage boys. As a result of its low prevalence, there have been few studies of its treatment and impact on survival. Here, we report the case of a patient who was refractory to multiple drugs but remains stable with trabectedin. To our knowledge, this is the first published report that supports antitumoral activity of trabectedin in DSRCT. Content Type Journal ArticleDOI 10.1007/s12032-010-9687-9Authors Ana López-González, Medical Oncology, Hospital Universitario Puerta de Hierro, Madrid, SpainBlanca Cantos, Medical Oncology, Hospital Universitario Puerta de Hierro, Madrid, SpainEva Tejerina, Pathology Anatomy, Hospital Universitario Puerta de Hierro, Madrid...

A t (11; 22) (p13; q12) EWS-WT 1 positive desmoplastic small round cell tumor of the maxilla: An unusual case indicating the role of molecular diagnosis in round cell sarcomas

Journal of Postgraduate Medicine — Tue, 24 Aug 2010 08:25:15 +0100

B Rekhi, R Basak, SB Desai, NA JambhekarJournal of Postgraduate Medicine 2010 56(3):201-205A desmoplastic small round cell tumor (DSRCT) is an uncommon tumor characterized by polyphenotypic expression and a specific reciprocal translocation t (11; 22) (p13; q12). It has been rarely identified in the head and neck region. Herein, we describe a DSRCT in the maxilla of a young man, who was initially diagnosed with a primitive neuroectodermal tumor (PNET), based on histopathological appearance of a round cell tumor, with MIC2 and -FLI-1 positivity, on immunohistochemistry (IHC). Diagnosis of a DSRCT was confirmed on molecular analysis with positive -RT-PCR and sequencing results for EWS-WT1 transcript and negativity for EWS-FL1. The case is presented to highlight the value of molecular diagnos...

Primary Desmoplastic Small Round Cell Tumor of Urinary Bladder [DIAGNOSIS IN ONCOLOGY]

Journal of Clinical Oncology — Thu, 17 Jun 2010 22:02:13 +0100

(Source: Journal of Clinical Oncology)

Desmoplastic Small Round-Cell Tumor: An Adult with Previous Exposure to Agent Orange

Japanese Journal of Clinical Oncology — Thu, 27 May 2010 16:15:24 +0100

Desmoplastic small round-cell tumor is an uncommon, highly aggressive tumor with a predilection for pediatric age groups and young adults. It is very unusual in the elderly population. Although Agent Orange has been associated with soft-tissue sarcoma, an association with desmoplastic small round-cell tumor has not been reported. A 52-year-old male presented with abdominal distention, dyspnea, and a 9 kg weight loss. Prior history was significant for hepatitis C and diabetes. He was a Vietnam veteran and he admitted being exposed to Agent Orange. On physical examination, the abdomen was distended and tense. Computed tomography scan of the chest, abdomen and pelvis demonstrated extensive mediastinal and retroperitoneal adenopathy, diffuse omental masses and extensive pleural, intra-abdomina...

Abdominal desmoplastic small round cell tumor of childhood: case report

Jornal Brasileiro de Patologia e Medicina Laboratorial — Fri, 21 May 2010 13:53:51 +0100

We report a case of DSRCT affecting an 11-year-old male patient, with intra-abdominal involvement characterized by a large retroperitoneal mass in left hypochondrium. Histological examination of the mass showed the presence of clusters of small blue round tumor cells surrounded by a dense desmoplastic stroma. Immunohistochemical analysis disclosed a positive reaction to desmin, WT-1 and cytokeratins. After diagnosis, the patient underwent chemo radiotherapy treatment, but died at the 24th month of follow-up. (Source: Jornal Brasileiro de Patologia e Medicina Laboratorial)

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Surgical Procedure Offers New Option For Pediatric Patients With Rare Cancer In Abdomen

Health News from Medical News Today — Fri, 21 May 2010 08:00:00 +0100

A study by a pediatric surgical oncologist from The University of Texas MD Anderson Children's Cancer Hospital shows that an adult surgery adapted for use in young patients increased the survival of children with rare tumors in the abdomen. The study, reported in the May 20 issue of the Journal of Pediatric Surgery, looked retrospectively at 24 pediatric patients diagnosed with a rare and aggressive pediatric cancer known as desmoplastic small round cell tumor (DSRCT)... (Source: Health News from Medical News Today)

Novel treatment for desmoplastic small round cell tumor: hyperthermic intraperitoneal perfusion

Journal of Pediatric Surgery — Fri, 30 Apr 2010 23:00:00 +0100

Conclusion: Hyperthermic intraperitoneal chemotherapy is safe in children with DSRCT. It may prolong disease-free survival in selected cases of DSRCT. It may have a limited role as an adjunct to local control in patients with DSRCT. (Source: Journal of Pediatric Surgery)

Desmoplastic small round cell tumor in the ovary: Report of two cases and literature review

Journal of Obstetrics and Gynaecology Research — Thu, 25 Mar 2010 00:00:00 +0100

We report two cases of DSRCT in young women that presented clinically as ovarian tumor with extensive pelvic and abdominal dissemination. Both patients underwent debulking surgery and combined chemotherapy. After primary therapy, the tumors recurred and both women died of the disease. The clinical presentation and differential diagnosis, as well as the treatment, including surgical debulking and combined chemotherapy are discussed. (Source: Journal of Obstetrics and Gynaecology Research)

Reverse transcriptase-polymerase chain reaction as an ancillary molecular technique in the diagnosis of small blue round cell tumors by fine-needle aspiration cytology.

American Journal of Clinical Pathology — Thu, 18 Mar 2010 15:40:14 +0100

Authors: Gautam U, Srinivasan R, Rajwanshi A, Bansal D, Marwaha RK, Vasishtha RK We evaluated the feasibility and usefulness of reverse transcriptase-polymerase chain reaction (RT-PCR) on fine-needle aspirates for categorization of small blue round cell tumors (SBRCTs). A total of 51 cases, including 25 Ewing sarcoma/peripheral primitive neuroectodermal tumors (PNETs), 11 rhabdomyosarcomas, 13 neuroblastomas, and 2 desmoplastic small round cell tumors (DSRCTs) were analyzed. The detection of the EWS-FLI1 (20/25) and EWS-ERG (4/25) fusion transcripts resolved 24 of 25 cases of Ewing sarcoma/PNET. The PAX3/7-FKHR fusion transcript was detected in 2 of 4 cases of alveolar rhabdomyosarcoma and the EWS-WT1 transcript in both cases of DSRCT. Tyrosine hydroxylase and 3,4-dihydroxyphenylalanin...

Cytokeratin (CK8/CK18) is not derived from CAM5.2 clone; and anticytokeratin CAM5.2 (Becton Dickinson) is not synonymous with the antibody (CK8/CK18)—reply

Human Pathology — Mon, 15 Mar 2010 15:18:13 +0100

The CAM5.2 antibody (Becton Dickinson) that we have been using came with an insert stating explicitly that this is a “monoclonal antibody test for analysis of cytokeratin proteins 8 and 18.” We thank Han et al for drawing our attention to a revision in the stated reactivities of the antibody by the manufacturer to cytokeratins 7 and 8 instead of cytokeratins 8 and 18. This revision, however, does not alter the interpretation or conclusion of our study that the desmoplastic small round cell tumor of the salivary gland was immunoreactive for cytokeratin. (Source: Human Pathology)

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[Paratesticular desmoplastic small round cell tumour: Case report with literature review.]

Cancer Radiotherapie — Thu, 25 Feb 2010 00:00:00 +0100

We report the case of a 27-year-old male who consulted on a progressive enlargement of the right hemiscrotum. He referred no previous urological symptoms and had no systemic symptomatology. Physical examination revealed a round elastic firm 1-cm mass of the epididymis, which was excised. A computed tomography scan showed a para-aortic mass of 1cm. Histological and immunohistochemical diagnosis confirmed a desmoplastic small round cell tumor. The patient received chemotherapy. Today, 6 months after diagnosis the patient remains well and free of disease. Recent reviews on desmoplastic small round cell tumors affecting the paratesticular area have shown a better prognosis for tumors of this origin compared to abdominal ones. We should include this lesion among the differential diagnosis of pa...

Desmoplastic small round cell tumour: an unusual presentation of an unusual tumour.

Med J Aust — Mon, 01 Feb 2010 00:00:00 +0100

Authors: Okera M, Moffat D, Selva-Nayagam S PMID: 20121690 [PubMed - in process] (Source: Med J Aust)

Temsirolimus for metastatic desmoplastic small round cell tumor

Pediatric Blood and Cancer — Fri, 01 Jan 2010 00:00:00 +0100

(Source: Pediatric Blood and Cancer)

Intra-abdominal desmoplastic small round cell tumour in a 39-year-old man

Clinical and Translational Oncology — Thu, 03 Dec 2009 18:25:00 +0100

We report the case of a 39 year-old man and briefly summarize the evidence about this tumor. Content Type Journal ArticleCategory Case ReportsDOI 10.1007/s12094-009-0442-7Authors Álvaro Pinto Marín, Hospital Universitario La Servicio de Oncología Médica Paz Paseo de la Castellana, 261 ES-28046 Madrid SpainMaría Garrido Arévalo, Hospital Universitario La Servicio de Oncología Médica Paz Paseo de la Castellana, 261 ES-28046 Madrid SpainAndrés Redondo Sánchez, Hospital Universitario La Servicio de Oncología Médica Paz Paseo de la Castellana, 261 ES-28046 Madrid SpainEnrique Espinosa Arranz, Hospital Universitario La Servicio de Oncología Médica Paz Paseo de la Castellana, 261 ES-28046 Madrid SpainPilar Zamora Auñón, Hospital Universitario La Servicio de Oncología Médica...

Metastatic retinoblastoma presenting as a left shoulder soft tissue mass: FNA findings and review of the literature

Diagnostic Cytopathology — Tue, 24 Nov 2009 00:00:00 +0100

Retinoblastoma is a relatively rare malignant pediatric tumor accounting for [sim]3% of childhood cancers and 1% of all cancer deaths in children under 15 years of age. During the clinical course of the disease, a metastasis usually occurs within the first year of diagnosis and is seen in 2% of retinoblastoma patients. Metastases to the intracranial region are common and account for [sim]50% of the metastatic cases. Metastasis to the soft tissue is very rare. Herein, we report a case of metastatic retinoblastoma presenting as a left shoulder soft tissue mass in a 14-year-old female with a 14-year history of familial bilateral retinoblastoma status post radiation therapy. In our case, the FNA cytology shows some features of the small round blue cell tumor group with inconspicuous Flexner-Wi...

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Soft tissue tumors associated with EWSR1 translocation

Virchows Archiv — Thu, 19 Nov 2009 20:00:48 +0100

Abstract The Ewing sarcoma breakpoint region 1 (EWSR1; also known as EWS) represents one of the most commonly involved genes in sarcoma translocations. In fact, it is involved in a broad variety of mesenchymal lesions which includes Ewing's sarcoma/peripheral neuroectodermal tumor, desmoplastic small round cell tumor, clear cell sarcoma, angiomatoid fibrous histiocytoma, extraskeletal myxoid chondrosarcoma, and a subset of myxoid liposarcoma. The fusion products between EWSR1 and partners usually results in fusion of the N-terminal transcription-activating domain of EWSR1 and the C-terminal DNA-binding domain of the fusion partner, eventually generating novel transcription factors. EWSR1 rearrangement can be visualized by the means of fluorescence in situ hybridization (FISH)...

Desmoplastic small round cell tumor of the submandibular gland—a rare but distinctive primary salivary gland neoplasm

Human Pathology — Mon, 16 Nov 2009 00:00:00 +0100

We report a case presenting as a primary submandibular gland tumor in a 24-year-old man. Histologically, although there were irregular tumor islands lying in an abundant desmoplastic stroma, there were also areas comprising large cellular islands with scanty stroma in between, raising the differential diagnosis of various salivary gland carcinomas. The tumor cells were medium sized, with hyperchromatic nuclei and moderate amounts of cytoplasm. The diagnosis of desmoplastic small round cell tumor was confirmed by the presence of a polyphenotypic immunoprofile (positive for cytokeratin, desmin, and neuron-specific enolase) and the characteristic EWS-WT1 gene fusion. Although rare, desmoplastic small round cell tumor has to be considered in the differential diagnosis of poorly differentiated ...

No effect of irinotecan in patients with resistant desmoplastic small round cell tumor

Pediatric Blood and Cancer — Wed, 28 Oct 2009 00:00:00 +0100

No Abstract. (Source: Pediatric Blood and Cancer)

Pitfalls in the differential diagnosis of renal tumor in an adolescent

Pediatric Blood and Cancer — Thu, 22 Oct 2009 23:00:00 +0100

We describe an 11-year-old female with a primary intra-renal mass. Initial differential diagnoses included primitive neuroectodermal tumor (PNET), desmoplastic small round cell tumor (DSRCT), and Wilms Tumor (WT). Extensive pathologic and molecular analysis on initial and relapsed tumor samples confirmed WT. The EWS-WT1 and EWS-FLI1 rearrangements, distinctive of DSRCT and PNET were negative. The differential diagnosis on monophasic blastemal WT may be complex. Primary renal DSRCT and PNET have been rarely described. Nevertheless, molecular confirmation for these rare conditions may be necessary in selected cases. Pediatr Blood Cancer © 2009 Wiley-Liss, Inc. (Source: Pediatric Blood and Cancer)

Sequential intensified chemotherapy with stem cell rescue for children and adolescents with desmoplastic small round-cell tumor

Bone Marrow Transplantation — Sun, 04 Oct 2009 23:00:00 +0100

Authors: G Bisogno, A Ferrari, A Rosolen, R Alaggio, G Scarzello, A Garaventa, G Arcamone & M Carli (Source: Bone Marrow Transplantation)

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Retroperitoneal desmoplastic small round cell tumor: pediatric patient treated with multimodal therapy.

World Journal of Gastroenterology : WJG — Fri, 04 Sep 2009 08:42:10 +0100

In this report, we describe the case of an 8-year-old boy diagnosed with DSRCT located in the retroperitoneal space. The patient has undergone surgical resection and adjuvant chemoradiation therapy, and is currently alive without disease recurrence. PMID: 19725162 [PubMed - in process] (Source: World Journal of Gastroenterology : WJG)

Evaluation of pediatric abdominal masses by fine-needle aspiration cytology: A clinicoradiologic approach

Diagnostic Cytopathology — Sun, 16 Aug 2009 23:00:00 +0100

The pathologist forms a very important part of the clinical team in the management of pediatric intra-abdominal masses in giving a rapid, accurate diagnosis for these potentially curable tumors. Fine-needle aspiration cytology (FNAC) is an invaluable tool in this regard when interpreted with clinicoradiologic parameters. With this in mind, we decided to evaluate the role of FNAC in pediatric abdominal masses in our institution. A total of 83 of 105 FNAC accessioned in the pathology department over 5 years (2003-2007) were studied. These included only cases where a diagnosis could be offered on cytology. Detailed clinicoradiological features were obtained from hospital records. Cytomorphological features examined included cellularity, architectural pattern, background, key cellular details....

Long-term remission after CD34+-selected PBSCT in a patient with advanced intra-abdominal desmoplastic small round-cell tumor

Bone Marrow Transplantation — Thu, 30 Jul 2009 23:00:00 +0100

Long-term remission after CD34+-selected PBSCT in a patient with advanced intra-abdominal desmoplastic small round-cell tumor Bone Marrow Transplantation advance online publication, August 31, 2009. doi:10.1038/bmt.2009.226 Authors: L Houet, I Möller, M Engelhardt, G Köhler, H Schmidt, D Herchenbach, M Schnitzler, A Schmitt-Graeff, A A Jungbluth, R Mertelsmann, B Rumstadt & C F Waller (Source: Bone Marrow Transplantation)

[Sarcomas] Multicenter Phase II Trial of Sunitinib in the Treatment of Nongastrointestinal Stromal Tumor Sarcomas

Journal of Clinical Oncology — Sun, 28 Jun 2009 23:00:00 +0100

Conclusion Sunitinib demonstrated notable evidence of metabolic response in several patients with non-GIST sarcoma. The relevance of disease control observed in subtypes with an indolent natural history is unknown, however, the durable disease control observed in DSRCT, solitary fibrous tumor, and giant cell tumor of bone suggests that future evaluation of sunitinib in these subtypes may be warranted. (Source: Journal of Clinical Oncology)

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Multicenter Phase II Trial of Sunitinib in the Treatment of Nongastrointestinal Stromal Tumor Sarcomas [Sarcomas]

Journal of Clinical Oncology — Sun, 28 Jun 2009 23:00:00 +0100

Outpatient and Home Chemotherapy with Novel Local Control Strategies in Desmoplastic Small Round Cell Tumor

Sarcoma — Tue, 09 Jun 2009 15:10:54 +0100

This report illustrates novel chemotherapy and local control interventions in a 5-year old patient. The patient was treated in the outpatient setting, achieved remission, with excellent quality of life. The patient presented with massive ascites and >1000 abdominal tumors. Neoadjuvant chemotherapy included vincristine (1.5 mg/m2), ifosfamide (3 g/m2/day×3), dexrazoxane/doxorubicin (750/75 mg/m2), and etoposide (150 mg/m2). Continuous hyperthermic peritoneal perfusion (CHPP) with cisplatin (100 mg/m2) was given after extensive cytoreductive surgery. This was followed by irinotecan (10 mg/m2/day×5×2 weeks) + temozolomide monthly × 2, then abdominal radiation 30 Gy with simultaneous temozolomi...

Friend leukemia virus integration-1 (FLI-1) expression in gastrointestinal stromal tumors.

The Turkish Journal of Gastroenterology — Sun, 31 May 2009 23:00:00 +0100

CONCLUSIONS: Friend leukemia virus integration-1 can be expressed in a variety of tumors, and is helpful in making the diagnosis of Ewing's sarcoma/primitive neuroectodermal tumor. We think that this protein is not expressed in gastrointestinal stromal tumors and it is not a part of the pathogenesis of this disease. PMID: 19530039 [PubMed - in process] (Source: The Turkish Journal of Gastroenterology)

Desmoplastic small round cell tumor in children: a new therapeutic approach

Journal of Pediatric Surgery — Fri, 01 May 2009 04:00:00 +0100

Conclusion: The patients DSRCT are sensitive to an aggressive combination of chemotherapy, surgical debulking, and radiation therapy, followed by autologous BMT. It appears that this new multifaceted treatment offers good palliation, which may prolong survival and a possible cure. (Source: Journal of Pediatric Surgery)

[Desmoplastic small round cell tumor of the abdomen: CT findings and radiologic-pathologic correlation in 3 cases.]

Radiologia — Tue, 17 Mar 2009 04:00:00 +0100

We describe the CT findings of abdominal desmoplastic small round cell tumor (DSRCT) in three patients. The most common imaging finding was the presence of single or multiple soft-tissue density intraperitoneal masses without apparent origin in an abdominal organ. In the first patient, a single peritoneal mass was located in the mesentery between the stomach and pancreas. In the second patient, an intraperitoneal pelvic mass was seen in the retrovesical space. In the third patient, a large homogeneous soft-tissue mass that nearly filled the entire peritoneal space was found. Two patients had multiple liver metastases and adenopathies at the time of diagnosis. The diagnosis was confirmed with CT-guided percutaneous biopsies in all three cases. DSRCT should be suspected in young men with mul...

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Desmoplastic small round cell tumor of the central nervous system: report of two cases and review of the literature

Virchows Archiv — Thu, 05 Mar 2009 15:00:50 +0100

In conclusion, CNS DSRCT follows a similar aggressive course as do peritoneal examples. Although rare, DSRCT warrants consideration in the differential diagnosis of “malignant small blue cell tumors” of the CNS. Content Type Journal ArticleCategory Original ArticleDOI 10.1007/s00428-009-0750-xAuthors Luciano Neder, Mayo Clinic Department of Laboratory Medicine and Pathology 200 First Street, SW Rochester MN 55905 USABernd W. Scheithauer, Mayo Clinic Department of Laboratory Medicine and Pathology 200 First Street, SW Rochester MN 55905 USAKeki E. Turel, Bombay Hospital Institute of Medical Sciences Department of Neurosurgery Mumbai IndiaMark A. Arnesen, Abbott Northwestern Hospital Department of Laboratory Medicine Minneapolis MN USARhett P. Ketterling, Mayo Clinic Department of ...

Potential Mesothelioma Treatment May Stop the Growth of Tumors

Asbestos and Mesothelioma News — Mon, 02 Mar 2009 15:54:45 +0100

A newly used cancer treatment has recently stopped the growth of aggressive sarcomas in two patients. One of the patients was diagnosed with malignant fibrous histiocytoma (MFH) and the other patient was diagnosed with desmoplastic small round cell tumor (DSRCT). After multiple surgeries and several different chemotherapy regimens had failed, the cancers were continuing to spread to other areas of their bodies. However, after just eight weeks of the NeoPlas Innovation’s treatment, scans revealed that both patients’ cancers had stopped spreading and some tumors had actually regressed. NeoPlas’ treatment is a combination of lovastatin and interferon. According to Dr. Stephen B. Cantrell, the NeoPlas Innovation Director of Research, “These are early results and not statistically valid...

[Small round cell desmoplastic tumour. Atypical morphology in the sub-maxillary gland]

Acta Otorrinolaringologica Espanola — Sun, 01 Mar 2009 00:00:00 +0100

We report a case of desmoplastic small round cell tumour of the sub-maxillary gland, with an evolution of 8 months, affecting a 36 year old male. He suffered chronic lymphatic leukaemia five years ago and needed a bone marrow transplant. There was a 4x3 cm tumour. There were no signs of malignancy on the CT scan. A right sub-maxillectomy was performed. The pathology analysis gave a diagnosis of DSRCT. Post-surgical radiotherapy was given. The definitive diagnosis was reached using immunohistochemical techniques, such as polyphenotypical differentiation (epithelial, mesenchymal and neural), and by demonstration of translocation (11;22)(p13;q12). Sub-maxillary location is very rare. PMID: 19401082 [PubMed - in process] (Source: Acta Otorrinolaringologica Espanola)

New Cancer Treatment Stops Aggressive Sarcomas

Cancercompass News: Other Cancer — Fri, 27 Feb 2009 05:00:00 +0100

NASHVILLE, Tenn. -- After all standard treatments had failed, a new cancer treatment has stopped the growth of aggressive sarcomas in two recent patients. These results are similar to the new protocol's previous success against several cancers, including melanoma, pancreatic, colon, mesothelioma and other sarcomas. One of the patients is an 18-year-old with malignant fibrous histiocytoma (MFH) that arose in the largest bone of his leg. The cancer spread throughout his lungs, despite aggressive surgery that replaced most of his leg with metal implants. The second patient was losing a battle with desmoplastic small round cell tumor (DSRCT), a very rare and aggressive cancer. After undergoing multiple surgical procedures a... (Source: Cancercompass News: Other Cancer)

Comparative evaluation of flow-cytometric immunophenotyping and immunocytochemistry in the categorization of malignant small round cell tumors in fine-needle aspiration cytologic specimens

Cancer Cytopathology — Wed, 17 Dec 2008 05:00:00 +0100

In this study, the authors evaluated the applicability of flow-cytometric immunophenotyping (FCI) and compared it with immunocytochemistry (ICC) for the accurate categorization of MSRCTs.In total, 37 consecutive MSRCTs that had been diagnosed with FNA cytology were analyzed by ICC and FCI using a panel of antibodies against desmin, vimentin, CD99/major histocompatibility class I-related antigen 2, neuron-specific enolase, and pancytokeratin. The final diagnoses included Ewing sarcoma (n = 17), rhabdomyosarcoma (n = 6; 4 embryonal and 2 alveolar subtypes), neuroblastoma (n = 10), desmoplastic small round cell tumor (n = 2), and retinoblastoma (n = 2).Accurate categorization was possible in 67.5% of cases by ICC and in 64.8% of cases by FCI. Concordant immunophenotyping results with either t...

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[Diagnosis in Oncology] Solitary Relapse of Desmoplastic Small Round Cell Tumor Detected by Positron Emission Tomography/Computed Tomography

Journal of Clinical Oncology — Thu, 16 Oct 2008 04:00:00 +0100

(Source: Journal of Clinical Oncology)

A new molecular variant of desmoplastic small round cell tumor: significance of WT1 immunostaining in this entity.

Human Pathology — Tue, 12 Aug 2008 04:00:00 +0100

Authors: Murphy AJ, Bishop K, Pereira C, Chilton-Macneill S, Ho M, Zielenska M, Thorner PS Desmoplastic small round cell tumor is a rare aggressive neoplasm, often presenting in young adult males. Although the classic features are well described, considerable clinical, pathologic, and immunohistochemical variation has been reported. The defining feature is a reciprocal translocation, t(11;22)(p13;q12), which fuses EWS on chromosome 22 to WT1 on chromosome 11. WT1 immunohistochemistry is reportedly useful in distinguishing desmoplastic small round cell tumor from other tumors. Herein, we describe a desmoplastic small round cell tumor of soft tissue with an unusual pattern of WT1 expression associated with a novel variant EWS-WT1 fusion transcript. We compare WT1 expression pattern with ...

Paediatric renal tumours: recent developments, new entities and pathological features.

Histopathology — Mon, 11 Aug 2008 04:00:00 +0100

Authors: Sebire NJ, Vujanic GM Paediatric renal tumours represent a relatively common group of childhood solid neoplasms, in which both diagnosis and treatment are highly dependent on the histopathological findings. In addition to Wilms' tumour (nephroblastoma), a number of specific distinct entities are now reported, including (congenital) mesoblastic nephroma, clear cell sarcoma of the kidney, rhabdoid tumour of the kidney, specific paediatric variants of renal cell carcinoma, and others such as renal primitive neuroectodermal tumour and desmoplastic small round cell tumour. Recent advances in both molecular biological findings and immunohistochemistry allow reliable diagnosis of most of these entities even on the basis of small needle biopsy specimens. This review highlights both th...

Fine needle biopsy and genetics, two allied weapons in the diagnosis, prognosis, and target therapeutics of solid pediatric tumors

Diagnostic Cytopathology — Fri, 01 Aug 2008 04:00:00 +0100

The recognition that genetic defects identify some pediatric solid tumors and may represent prognostic markers has provided cytologists with an extra tool for dealing with such tumors. Using some entities as archetypes, we discuss the importance of the association of fine needle biopsy and genetics, in the diagnosis, prognosis, and therapy selection of solid pediatric tumors. Immunocytochemistry is important to differentiate neuroblastoma, PNET/Ewing sarcoma, alveolar rhabdomyosarcoma, lymphoma, and desmoplastic small round cell tumor. Despite its usefulness in many cases, it is not conclusive and some of the aforementioned tumors even share the expression of some antibodies. The detection of specific diagnostic translocations will thus provide additional information and allows a precise c...

Imprint cytology of high-grade immature ovarian teratoma: A case report, literature review, and distinction from other ovarian small round cell tumors

Diagnostic Cytopathology — Thu, 10 Jul 2008 04:00:00 +0100

Immature ovarian teratoma (IOT) is a rare and aggressive malignant neoplasm characterized by immature neural tissue. The cytomorphologic features have only rarely been described. We herein describe an additional case and review the literature regarding this entity. To the best of our knowledge, this is the first reported case with imprint cytology. A 35-year-old woman presented with a pelvic mass which was resected and sent for frozen section evaluation. Imprint smears and frozen section of the mass were diagnostic of IOT. IOT has diagnostic cytologic features which show complete concordance with histology. Differential diagnoses include other small round cell neoplasms such as ovarian neuroblastoma, small cell carcinoma of hypercalcemic type, primitive neuroectodermal tumor, Wilm's tumor,...

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Desmoplastic small round cell tumor - abdomen.

Indian Journal of Pathology and Microbiology — Tue, 01 Jul 2008 04:00:00 +0100

Authors: Kinra P, Pujahari AK PMID: 18723992 [PubMed - in process] (Source: Indian Journal of Pathology and Microbiology)

Desmoplastic Small Round Cell Tumor: A Clinical, Pathological, and Immunohistochemical Study of 18 Chinese Cases

International Journal of Surgical Pathology — Sun, 22 Jun 2008 04:00:00 +0100

Desmoplastic small round cell tumor (DSRCT) was first reported in 1989. Generally, DSRCT is considered to be an aggressive malignant neoplasm that mainly occurs in the abdominal cavity and has been often seen in adolescents and young male adults. In the present study, a total of 18 cases of DSRCT reported in China between October 1998 and June 2006, including one case treated by the authors, were reviewed and analyzed. Among them, 14 had tumors in the abdominal cavity; the other four cases had tumors in the left fossa orbitalis, the root of the tongue, the soft tissue behind the left eyeball, and the abdominal wall (umbilicus). Overall, the 1-year, 3-year, and 5-year survival rates were 52.36%, 27.92%, and 27.92%, respectively. The survival rate of DSRCT patients is disappointing; however,...

Intracerebral small round cell tumor: An unusual case with EWS-WT1 translocation

Pediatric Blood and Cancer — Mon, 16 Jun 2008 04:00:00 +0100

Desmoplastic small round cell tumor (DSRCT) is a rare tumor, seen both in children and young adults with a marked predilection for the peritoneal cavity. Histology showed a small round cell tumor with a fibromyxoïd stroma and immunohistochemistry indicated neural and mesenchymal differentiation, and diagnosis was made by molecular detection of the EWS-WT1 fusion gene product. DSRCT should be considered in the differential diagnosis of intracranial small round cell tumors. Pediatr Blood Cancer © 2008 Wiley-Liss, Inc. (Source: Pediatric Blood and Cancer)

Outpatient and Home Chemotherapy with Novel Local Control Strategies in Desmoplastic Small Round Cell Tumor

Sarcoma — Thu, 12 Jun 2008 00:43:59 +0100

[RSNA Education Exhibits] Neoplastic and Nonneoplastic Conditions of Serosal Membrane Origin: CT Findings

Radiographics recent issues — Wed, 14 May 2008 04:00:00 +0100

Computed tomography (CT) is an important imaging modality for diagnosis and follow-up of neoplastic or nonneoplastic conditions of the serosal membrane. The characteristic CT findings of malignant pleural mesothelioma include unilateral pleural effusion, thickening of the mediastinal pleura, and circumferential and nodular pleural thickening of greater than 1 cm. Malignant peritoneal mesothelioma manifests as a large mass or diffuse peritoneal thickening without a definable mass and is difficult to differentiate from peritoneal carcinomatosis or tuberculosis. The imaging features of primary serous papillary carcinoma of the peritoneum resemble those of peritoneal carcinomatosis; however, the ovary is usually of normal size. The possibility of desmoplastic small round cell tumor should be c...

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Primary desmoplastic small round cell tumor of the femur

Skeletal Radiology — Sat, 10 May 2008 09:14:22 +0100

We report a 10-year-old girl with primary DSRCT arising within the left femur. The patient presented with knee pain, and radiological findings were strongly suggestive of osteogenic sarcoma. In addition to the typical microscopic appearance and immunophenotype, RT-PCR demonstrated the chimeric transcript of EWS-WT1, which is diagnostic of DSRCT. Pulmonary metastases were present at initial staging studies, but no abdominal or pelvic lesion was present. Despite chemotherapy and complete tumor excision, the patient developed progressive lung and bone metastases and died 3 years after initial presentation. This is the second reported case of primary DSRCT of bone with genetic confirmation. Content Type Journal ArticleCategory Case ReportDOI 10.1007/s00256-008-0501-0Authors Akihiko Y...

[AFIP Archives] From the Archives of the AFIP: Primary Peritoneal Tumors: Imaging Features with Pathologic Correlation

Radiographics recent issues — Tue, 18 Mar 2008 04:00:00 +0100

Primary peritoneal tumors are uncommon lesions that arise from the mesothelial or submesothelial layers of the peritoneum. Primary malignant mesothelioma, multicystic mesothelioma, primary peritoneal serous carcinoma, leiomyomatosis peritonealis disseminata, and desmoplastic small round cell tumor are the most prominent of these rare lesions. Primary malignant mesothelioma is a highly aggressive malignancy that occurs most commonly in older men and that has a strong association with high levels of asbestos exposure. It manifests most often as diffuse sheetlike or nodular thickening of the peritoneal surfaces, but it may occasionally be a localized mass. Multicystic mesothelioma occurs most frequently in women and has benign or indolent biologic behavior in the majority of patients. It is a...

Significant clinical benefit of first-line palliative chemotherapy in advanced soft-tissue sarcoma

Cancer — Fri, 15 Feb 2008 05:00:00 +0100

The efficacy of palliative chemotherapy was investigated in a large group of patients with advanced soft-tissue sarcomas (STS) treated on routine palliative protocols.Patients with STS who had first-line chemotherapy for advanced and/or metastatic disease between 1991 and 2005 were identified from the Royal Marsden Hospital's sarcoma database. Patients with Ewing sarcoma, rhabdomyosarcoma, desmoplastic small round cell tumor, and gastrointestinal stromal tumors were excluded from the study.In all, 488 patients (242 male, 246 female) fulfilled the study criteria. The median age was 49 years and the majority (83%) received chemotherapy for metastatic disease. The most common histologic subtypes were leiomyosarcoma (35%) synovial sarcoma (13%), liposarcoma (10%), and malignant fibrous histioc...

Desmoplastic small round cell tumour: A review of literature and treatment options.

Surgical Oncology — Wed, 09 Jan 2008 05:00:00 +0100

CONCLUSION: Surgical excision is only recommended for non-metastatic disease with combination chemo-radiotherapy as an adjunct. These modalities used in isolation may have less impact. Furthermore, the side effect profile from radiotherapy may outweigh any survival benefit. For advanced disease, symptom control is most important as these modalities impact survival minimally and palliation of secondary symptoms is paramount. Multi-disciplinary team and specialist centre review for histology and oncology are essential in managing this disease process and will enable greater numbers of patients to be enrolled into therapeutic trials and future evolving therapies. PMID: 18191563 [PubMed - as supplied by publisher] (Source: Surgical Oncology)

Desmoplastic Small Round Cell Tumor (DSRCT) With Ovarian Involvement in 2 Young Women.

Applied Immunohistochemistry & Molecular Morphology — Tue, 25 Dec 2007 01:22:49 +0100

Page: 94DOI: 10.1097/01.pai.0000213158.71934.9cAuthors: Fang, Xueping MD *; Rodabaugh, Kerry MD +; Penetrante, Remedios MD ++; Wong, Michael MD, PhD [S]; Wagner, Timothy MD [//]; Sait, Sheila PhD + [sharp]; Mhawech-Fauceglia, Paulette MD + (Source: Applied Immunohistochemistry & Molecular Morphology)

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Desmoplastic small round cell tumor of the lung: case report.

Chinese Medical Journal — Thu, 20 Dec 2007 05:00:00 +0100

Authors: Wang ZM, Xiao WB, Zheng SS PMID: 18167230 [PubMed - in process] (Source: Chinese Medical Journal)

Desmoplastic small round cell tumor masquerading as advanced ovarian cancer

International Journal of Gynecological Cancer — Fri, 14 Dec 2007 17:18:40 +0100

International Journal of Gynecological Cancer, Volume 0, Issue 0, Page ???, OnlineEarly Articles. Bland AE, Shah AA, Piscitelli JT, Bentley RC, Secord AA. Desmoplastic small round cell tumor masquerading as advanced ovarian cancer. Int J Gynecol Cancer 2008.Desmoplastic small round cell tumor (DSRCT) is a rare abdominal malignancy usually diagnosed in ... (Source: International Journal of Gynecological Cancer)

Mesenchymal neoplasms of the major salivary glands: clinicopathological features of 18 cases

European Archives of Oto-Rhino-Laryngology — Sat, 13 Oct 2007 17:13:32 +0100

We describe the clinico-pathologic features of 18 cases of mesenchymal neoplasms of the major salivary glands experienced at Asan Medical Center, Seoul, Korea, from 1998 to 2004. Mesenchymal neoplasms accounted for 3.4% of the total of 524 major salivary gland tumors. The parotid gland was the preponderant site (15 cases). Thirteen tumors were benign, constituting 3.5% of the total of 371 benign neoplasms. Schwannomas were the most common benign tumors (six cases), followed by lipomas (three cases), plexiform neurofibroma, hemangioma, desmoid tumor, and solitary fibrous tumor (one each). The malignant tumors consisted of one dermatofibrosarcoma protuberans, synovial sarcoma, leiomyosarcoma, pleomorphic liposarcoma and desmoplastic small round cell tumor each. Immunohistochemical anal...

Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical and molecular study of four patients.

J Formos Med Assoc — Mon, 01 Oct 2007 04:00:00 +0100

Conclusion:</strong> DSRCT is an aggressive tumor that usually occurs in the peritoneum of young males. The tumor is characterized by the expression of cytokeratin, vimentin, desmin and WT-1. RT-PCR, using paraffin-embedded tissue sections, can effectively detect the characteristic <em>EWS-WT1</em> fusion gene transcript. PMID: 17964965 [PubMed - in process] (Source: J Formos Med Assoc)

[Diagnosis in Oncology] Desmoplastic Small Round Cell Tumor of Meckels Diverticulum

Journal of Clinical Oncology — Mon, 30 Jul 2007 04:00:00 +0100

(Source: Journal of Clinical Oncology)

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Desmoplastic small round cell tumor: Using FISH as an ancillary technique to support cytologic diagnosis in an unusual case

Diagnostic Cytopathology — Wed, 18 Jul 2007 04:00:00 +0100

Desmoplastic small round cell tumor is a rare and aggressive neoplasm that predominantly affects young males. In almost all cases, a reciprocal translocation is present resulting in the fusion of the Ewing sarcoma gene with the Wilms' tumor gene. Here we describe an unusual case occurring in a 59-year-old male, in which fluorescence in situ hybridization (FISH) was used in conjunction with immunohistochemical studies to confirm the diagnosis. To our knowledge, this is the first reported case of using FISH as an ancillary technique to confirm the cytologic diagnosis of this tumor. Diagn. Cytopathol. 2007;35:516-520. © 2007 Wiley-Liss, Inc. (Source: Diagnostic Cytopathology)

[Original articles] Friend leukaemia integration-1 expression in malignant and benign tumours: a multiple tumour tissue microarray analysis using polyclonal antibody

Journal of Clinical Pathology — Fri, 08 Jun 2007 04:00:00 +0100

Conclusion: This study was the first to show that FLI-1 can be seen in a variety of solid tumours, some of which had never been explored before. This finding should be kept in mind, especially when using FLI-1 as a marker for finding the primary origin of poorly differentiated metastatic tumour. Finally, despite the expression of FLI-1 in numerous malignancies, it is still considered to be highly sensitive and specific in distinguishing EWS/PNET from other tumour types in general and from other SRCTs in particular. (Source: Journal of Clinical Pathology)

From Krukenberg to Today: The Ever Present Problems Posed by Metastatic Tumors in the Ovary. Part II

Advances in Anatomic Pathology — Mon, 30 Apr 2007 23:00:00 +0100

This is the second of a two-part consideration of metastatic tumors to the ovary. Here, the matter is considered in 16 categories, largely site-specific. The first tumor discussed is gastric carcinoma of intestinal-type whose ovarian manifestations have been the subject of a recent paper which emphasized its differences from the Krukenberg tumor. Coverage of intestinal adenocarcinoma emphasizes the landmark 1987 paper of RH Lash and WR Hart. The section on pancreatic neoplasms reemphasizes the problems caused by metastatic ductal carcinoma, considered primarily in Part I, and discusses less common issues such as spread of neuroendocrine and acinar cell carcinomas. The limited information on spread of tumors of the gallbladder and extrahepatic bile ducts is then reviewed before more detaile...

[Diagnosis in Oncology] Desmoplastic Small Round-Cell Tumor of the Pancreas

Journal of Clinical Oncology — Fri, 06 Apr 2007 04:00:00 +0100

(Source: Journal of Clinical Oncology)

Hepatic metastasectomy in children

Cancer — Thu, 05 Apr 2007 04:00:00 +0100

There are little data regarding the safety and efficacy of hepatic metastasectomy for solid tumors in childhood. We reviewed our institutional experience to assess operative mortality and morbidity, technique of resection, local control, and survival in pediatric patients undergoing liver resection for metastases.All pediatric patients who underwent hepatic resection for metastatic disease from August 1988 to July 2005 were retrospectively identified and clinical data were collected.Fifteen patients were identified during this period and primary malignancies included neuroblastoma (7), Wilms tumor (3), osteogenic sarcoma (2), malignant gastric epithelial tumor (1), and desmoplastic small round cell tumor (2). Twelve patients underwent anatomical hepatic resections and 3 had wedge resection...

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Desmoplastic Small Round Cell Tumor of the Kidney in Childhood.

The American Journal of Surgical Pathology — Tue, 27 Mar 2007 08:59:05 +0100

(Source: The American Journal of Surgical Pathology)

[Atypical desmoplastic small round cell tumor in a very young child]

Annales de Pathologie — Thu, 01 Feb 2007 05:00:00 +0100

Authors: Berrebi D, Benkirane A, Ferkdadji L, de Lagausie P, Delattre O, Chomette P, Schleiermacher G, Michon J, Peuchmaur M PMID: 17568363 [PubMed - indexed for MEDLINE] (Source: Annales de Pathologie)

A phase II study of imatinib mesylate in children with refractory or relapsed solid tumors: A Children's Oncology Group study

Pediatric Blood and Cancer — Mon, 29 Jan 2007 07:00:00 +0100

Imatinib mesylate is a small molecule inhibitor of certain tyrosine kinases, most notably the chimeric bcr-abl fusion protein found in CML. It also inhibits KIT and PDGF receptor tyrosine kinases in vitro. Ewing sarcoma, osteosarcoma, neuroblastoma, desmoplastic small round cell, and synovial sarcomas often overexpress KIT or the PDGF receptor. A phase II study of imatinib in children and young adults with select solid tumors was performed.Patients less than 30 years of age with refractory or recurrent Ewing sarcoma, osteosarcoma, neuroblastoma, desmoplastic small round cell, synovial sarcomas or GIST were eligible. Imatinib was administered daily for 28 day courses at a dose of 440 mg/m2/day. Responses were assessed according to Response Evaluation Criteria in Solid Tumor (RECIST).Seventy...

Malignant small round cell tumor of the heart: a diagnostic dilemma.

Cardiovascular Pathology — Mon, 01 Jan 2007 05:00:00 +0100

We report a rare malignant small round cell tumor of the heart in a 26-year-old woman. She had been symptomatic 15 days after vaginal delivery. Immunohistochemistry revealed divergent differentiation; hence, the tumor was designated as desmoplastic small round cell tumor. This is the first report of such a tumor in the heart. PMID: 17218216 [PubMed - indexed for MEDLINE] (Source: Cardiovascular Pathology)

Cytology of desmoplastic small round-cell tumor

Cancer Cytopathology — Fri, 15 Dec 2006 07:00:00 +0100

This study was designed to compare prechemotherapy and postchemotherapy cytomorphology of DSRCT and to evaluate for distinct chemotherapy-induced changes.The authors searched their respective institutional databases for all DSRCT cases with an associated FNAB. FNAB slides, immunocytochemistry, and cytogenetic results were reviewed.Six aspirates from 5 patients were identified, 3 of which were postchemotherapy. The postchemotherapy cases demonstrated cytologic findings not typically described in DSRCTs, including prominent and conspicuous nucleoli, discohesive single-cell architecture, and slightly larger cell size.Cytomorphologic variability was prominent in prechemotherapy cases, and no case could be classified as DSRCT on cytology alone; immunohistochemistry was necessary for definitive ...

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[Short reports] Coexpression of an unusual form of the EWS-WT1 fusion transcript and interleukin 2/15 receptor {beta}mRNA in a desmoplastic small round cell tumour

Journal of Clinical Pathology — Wed, 04 Oct 2006 06:00:00 +0100

Conclusion: The induction of the IL-2/15 receptor signalling pathway may contribute to tumorigenesis in DSRCT through a paracrine or an autocrine system, even though the EWS–WT1 was an unusual form. (Source: Journal of Clinical Pathology)

Sister Mary Joseph's nodule as presenting sign of a desmoplastic small round cell tumor

Pediatric Blood and Cancer — Tue, 26 Sep 2006 03:33:02 +0100

Umbilical metastases, also named Sister Mary Joseph's nodules, are well documented in the adult population and most often represent an underlying intra-abdominal malignancy, usually a carcinoma of gastrointestinal or gynecologic origin. They are indicative of widespread abdominal disease and are associated with a poor prognosis. An extensive review of the literature reveals only two such presentations in the pediatric population. A 14-year-old male presented with an umbilical mass, which was found to be a metastatic lesion of a desmoplastic small round cell tumor (DSRCT) of the abdomen. The diagnosis of an intra-abdominal malignancy, most commonly a DSRCT, should be considered in the presence of an umbilical mass in a child. Pediatr Blood Cancer © 2006 Wiley-Liss, Inc. (Source: Pediatric ...

Desmoplastic small round cell tumor in childhood: The St. Jude Children's Research Hospital experience

Pediatric Blood and Cancer — Tue, 26 Sep 2006 03:33:02 +0100

Desmoplastic small round cell tumor (DSRCT) is a rare, primarily intra-abdominal tumor that has a poor outcome with current therapies.We retrospectively reviewed patient characteristics, presenting symptoms, tumor pathology, treatment, and outcome of 11 pediatric patients with DSRCT at our institution.The cohort included 1 female and 10 male patients. Median age at diagnosis was 14 years (range 5-21 years). In eight (73%) patients, the primary tumor was abdominal or pelvic, and in one patient each, it was submental, mediastinal, and paratesticular. Nine (82%) patients had metastatic disease. All tumors showed polyphenotypic differentiation by immunohistochemistry. The EWS-WT1 transcript was detected in six of seven tumors tested. One tumor showed rhabdomyoblastic differentiation after ther...

Desmoplastic small round cell tumor of the pleura

Pediatric Blood and Cancer — Tue, 26 Sep 2006 03:33:02 +0100

Desmoplastic small round cell tumor (DSRCT) is a rare neoplasm with aggressive behavior. Usually it presents as a peritoneal mass, although other cases in various locations have been described. Since less than 10 cases of primary DSRCT in the pleura have been described, it is of interest to report a pediatric case arising from the pleura. The diagnosis was confirmed by molecular detection of the EWS/WT-1 fusion gene product. Multidisciplinary treatment with chemotherapy, radiotherapy, and surgical resection resulted in a progression-free survival time above the median survival, suggesting that this conventional approach could prove effective for this rare and very aggressive malignancy. Pediatr Blood Cancer © 2006 Wiley-Liss, Inc. (Source: Pediatric Blood and Cancer)

Response to vinorelbine and low-dose cyclophosphamide chemotherapy in two patients with desmoplastic small round cell tumor

Pediatric Blood and Cancer — Tue, 26 Sep 2006 03:33:02 +0100

We report two cases of abdominal desmoplastic small round cell tumor (DSRCT) that showed a clinical response to the vinorelbine/low-dose cyclophosphamide combination that has been claimed to be effective for rhabdomyosarcoma. This observation may prompt further investigation into the activity of such a regimen in DSRCT patients with recurrent or refractory disease, with a view to a possible future role as maintenance therapy in controlling minimal residual disease in patients who achieve complete remission with intensive induction multimodality therapy. Pediatr Blood Cancer © 2005 Wiley-Liss, Inc. (Source: Pediatric Blood and Cancer)

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Desmoplastic small round cell tumour : Cytological and immunocytochemical features

CytoJournal — Fri, 18 Mar 2005 00:00:00 +0100

Conclusion: The use of an adjunct immunocytochemical panel coupled with the cytomorphological characteristics allows the diagnosis of DSRCT in cytological specimens. (Source: CytoJournal)