MedWorm: Chordoma

[Review] Chordoma: current concepts, management, and future directions

The Lancet Oncology — Wed, 01 Feb 2012 05:00:00 +0100

Chordoma is a rare bone cancer that is aggressive, locally invasive, and has a poor prognosis. Chordomas are thought to arise from transformed remnants of notochord and have a predilection for the axial skeleton, with the most common sites being the sacrum, skull base, and spine. The gold standard treatment for chordomas of the mobile spine and sacrum is en-bloc excision with wide margins and postoperative external-beam radiation therapy. Treatment of clival chordomas is unique from other locations with an enhanced emphasis on preservation of neurological function, typified by a general paradigm of maximally safe cytoreductive surgery and advanced radiation delivery techniques. (Source: The Lancet Oncology)

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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...

Endoscopic management of a lower clival chondroid chordoma: case report.

Turkish Neurosurgery — Mon, 30 Jan 2012 05:00:02 +0100

We report herein a case of lower clival chondroid chordoma, focusing on the surgical procedure of endoscopic endonasal surgery. A 36-yearold woman presented with progressive headache, right shoulder pain, and right hypoglossal nerve palsy. Computed tomography (CT) and magnetic resonance (MR) imaging revealed an extradural tumor located in the lower clivus, including the anterior aspect of the foramen magnum, deeply compressing the medulla and upper cervical spinal cord. Endoscopic endonasal surgery was performed via two nostrils. Since the basiocciput was destroyed by the tumor, removal of the tumor allowed identification of the middle clivus superiorly, the anterior arch of the atlas inferiorly, and anteromedial parts of occipital condyles bilaterally without drilling ...

Editorial: Chordoma model.

Journal of Neurosurgery — Fri, 27 Jan 2012 05:00:00 +0100

Authors: Park DM PMID: 22283190 [PubMed - as supplied by publisher] (Source: Journal of Neurosurgery)

Characterization of cancer stem-like cells in chordoma.

Journal of Neurosurgery — Fri, 27 Jan 2012 05:00:00 +0100

Conclusions Cancer stem-like cell detection might be an important step in determining the recurrent and metastatic characteristics of chordoma. This finding may lead to the development of new approaches toward treatments of chordomas. PMID: 22283189 [PubMed - as supplied by publisher] (Source: Journal of Neurosurgery)

Establishment and characterization of a primary human chordoma xenograft model.

Journal of Neurosurgery — Fri, 27 Jan 2012 05:00:00 +0100

Conclusions To the best of their knowledge, the authors have established the first primary chordoma xenograft that will provide a useful preclinical model for this disease and a platform for therapeutic development. PMID: 22283186 [PubMed - as supplied by publisher] (Source: Journal of Neurosurgery)

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Intradural chordoma of the Meckel's cave: A challenging differential diagnosis

Neuropathology — Sat, 21 Jan 2012 00:31:02 +0100

Chordomas are midline tumors that arise from embryonic remnants of the notochord and are considered to be malignant tumors because of their tendency to invade and destroy the involved bone. Cases of intradural chordomas without bone involvement have been rarely described with a predilection for prepontine location. The absence of bony invasion renders the complete excision of these tumors more feasible and is related to their better prognosis in comparison to conventional chordomas. Herein we report the first intradural chordoma arising in the Meckel's cave. The intradural location of the lesion, outside midline structures, in the absence of bone infiltration, made the differential diagnosis versus other meningeal lesions such as chordoid meningioma challenging. The intense and strong immu...

[Complex Surgical Care of a Defect in the Pelvis after Repeated Curatively Intended Resections of a Rare Sacral Chordoma with Initially Inadequate Resection and Multiple Episodes of Recurrent Tumor Growth.]

Zentralblatt fur Chirurgie — Tue, 17 Jan 2012 05:00:00 +0100

Authors: Papaioannou P, Altmann S, Damert HG PMID: 22252377 [PubMed - as supplied by publisher] (Source: Zentralblatt fur Chirurgie)

Neurosurgery at Queen Square

Journal of Pakistan Medical Students — Thu, 29 Dec 2011 05:00:00 +0100

This article has been peer reviewed. Article Submitted on: 9th October 2011 Article Accepted on: 28th November 2011 Funding sources: None declared Correspondence to:Hamza Tariq Medical student Address:King Edward Medical University, Lahore, Pakistan. Email: hamxatariq@yahoo.com Download PDF Elective Report The day I finished studying the anatomy of brain, I knew that I wanted to pursue neurosurgery as a career speciality. A medical student getting fascinated by the mysterious workings of the human brain is not something unusual. Here, in a mere 3 pounds of living tissue, lies the complexity sufficient to incarnate and store the record of a lifetime of the richest human experience. Our brains contain the secrets of ourselves. Its mystery lies in the fact that it con...

Ectopic pituitary adenoma of the clivus presenting with apoplexy: case report and review of the literature.

Clinical Neuropathology — Mon, 26 Dec 2011 11:31:06 +0100

We report a 78-year-old male with known prostate carcinoma admitted with acute onset of blurred vision, suggestive of apoplexy. Work-up revealed unilateral cranial nerve VI palsy and neuroimaging showed a mass confined to the clivus; sellar region was normal. Preoperative considerations included chordoma, chondrosarcoma, or metastatic prostate carcinoma to bone. Resection was via endoscopic transsphenoidal approach to the clivus. An ectopic null cell pituitary adenoma with bland infarction was identified as the cause of the patient's clinical apoplexy. No antecedent precipitating factors for apoplexy were present; specifically the patient had not received leuprolide preoperatively, a known precipitant of pituitary apoplexy in prostate cancer patients who receive drug. We review the literat...

Neurosurgery at Queen Square

Journal of Pakistan Medical Students — Sat, 17 Dec 2011 05:00:00 +0100

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Endonasal endoscopic approach to clival and posterior fossa chordomas

Operative Techniques in Otolaryngology - Head and Neck Surgery — Thu, 01 Dec 2011 05:00:00 +0100

This article describes the “endoscopic binostril (transeptal/transeptal) approach” to resection of clival chordomas. (Source: Operative Techniques in Otolaryngology - Head and Neck Surgery)

Endoscopic versus open approaches to the skull base: A comprehensive literature review

Operative Techniques in Otolaryngology - Head and Neck Surgery — Thu, 01 Dec 2011 05:00:00 +0100

The surgical approach to the skull base has traditionally been transcranial, often involving extensive bone drilling, brain retraction, and nerve manipulation to expose pathology. The endoscopic endonasal approach represents a minimal access, maximally aggressive alternative that provides a direct route to the area of interest. Few data exist that can be used to compare these 2 surgical strategies. We conducted a systematic review of case series and case reports in hope of furthering our understanding of the role of endoscopy in the management of difficult cranial base lesions. We found that the endonasal endoscopic technique generates equivalent or greater rates of gross total resection than open approaches for craniopharyngiomas, clivalchordomas, odontoid resection, and tuberculumsellaem...

[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...

Treatment of chordoma with imatinib complicated by intracranial hemorrhage: a case showing dissociation between biological effect and therapeutic outcome

Journal of Neuro-Oncology — Fri, 25 Nov 2011 17:56:56 +0100

Content Type Journal ArticleCategory Letter to the EditorPages 1-3DOI 10.1007/s11060-011-0767-2Authors François Mercier, Massachusetts General Hospital, 185 Cambridge Street, Boston, MA 02114, USAMarie-Christine Guiot, Montreal Neurological Institute, Montreal, CanadaMichel W. Bojanowski, Centre Hospitalier de l’Universite de Montreal, Montreal, Canada Journal Journal of Neuro-OncologyOnline ISSN 1573-7373Print ISSN 0167-594X (Source: Journal of Neuro-Oncology)

Tumours of the atlas and axis: a 37-year experience with diagnosis and management

La Radiologia Medica — Fri, 18 Nov 2011 06:53:23 +0100

Conclusions Bone tumours of the cervical spine are rare. However, they should be kept in mind when examining patients with neck pain or neurological symptoms at the extremities. In most cases, only intralesional surgery can be administered. Combined radiation therapy and chemotherapy is indicated for certain tumour histologies. Content Type Journal ArticleCategory Musculoskeletal Radiology / Radiologia Muscolo-ScheletricaPages 1-20DOI 10.1007/s11547-011-0753-yAuthors A. F. Mavrogenis, Orthopaedic Oncology Service, Department of Orthopaedics, Istituto Ortopedico Rizzoli, University of Bologna, Via Di Barbiano 1/10, 40136 Bologna, ItalyG. Guerra, Orthopaedic Oncology Service, Department of Orthopaedics, Istituto Ortopedico Rizzoli, University of Bologna, Via Di Barbia...

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Parapharyngeal chordoma: A diagnostic challenge and potential mimic of pleomorphic adenoma on fine‐needle aspiration cytology

Diagnostic Cytopathology — Fri, 18 Nov 2011 05:00:00 +0100

We report the cytologic findings of a recurrent chordoma sampled through transoral FNA from the parapharyngeal space of a 66‐year‐old woman. As the prior history of chordoma was not available during the rapid onsite evaluation, the presence of bland epithelioid nonvacuolated cells and spindle cells intimately admixed with a fibrillary, intensely metachromatic material led to an initial diagnosis of pleomorphic adenoma. Review of the patient's prior pathology specimen and of the Papanicolaou‐stained smears and cellblock sections showing rare multivacuolated (physalipherous) cells led to the correct diagnosis, which was supported by immunoperoxidase stains (cytokeratin AE1/AE3+, S100+, GFAP−). A review of the literature found no previous instances in which chordomas mimicked pleomorp...

Endoscopic endonasal removal of laterally extended clival chordoma using side-viewing scopes

Acta Neurochirurgica — Tue, 15 Nov 2011 06:47:07 +0100

Conclusions Though a longer follow-up is needed to evaluate its effectiveness in long-term tumor control, the surgical maneuver using the side-viewing endoscope is effective for removing laterally extended clival chordomas. Content Type Journal ArticleCategory Technical NotePages 1-6DOI 10.1007/s00701-011-1225-9Authors Masaaki Taniguchi, Department of Neurosurgery, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, 650-0017 Hyougo, JapanEiji Kohmura, Department of Neurosurgery, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, 650-0017 Hyougo, Japan Journal Acta NeurochirurgicaOnline ISSN 0942-0940Print ISSN 0001-6268 (Source: Acta Neurochirurgica)

Primary intraosseous myoepithelioma arising in the iliac bone and displaying trisomies of 11, 15, 17 with del (16q) and del (22q11)-A rare case report with review of literature.

Pathology, Research and Practice — Wed, 02 Nov 2011 04:00:00 +0100

Authors: Rekhi B, Amare P, Gulia A, Baisane C, Patil A, Agarwal S, Puri A, Jambhekar NA Abstract Mixed tumors are uncommonly observed in the musculoskeletal system, where they form a common spectrum with a myoepithelioma and a parachordoma. Herein, we present a rare case of a mixed tumor/myoepithelioma arising in the iliac bone of a young adult male who presented with swelling in his right hip. Radiological imaging disclosed a large, intraosseous, lytic, heterogenous mass with a soft tissue component. Biopsy and subsequent tumor resection showed an 18cm sized tumor involving the iliac bone and soft tissues and comprising polygonal and spindly cells, arranged in cords and aggregates, embedded in a myxohyaline stroma with osteochondroid differentiation. Tumor cells exhibited mild nuc...

Wide resection of sacral chordoma via a posterior approach

International Orthopaedics — Fri, 28 Oct 2011 16:56:47 +0100

Conclusion Wide resection via the posterior approach and gauze packing technique could be used for management of sacral chordoma with acceptable results. Content Type Journal ArticleCategory Original PaperPages 1-6DOI 10.1007/s00264-011-1381-9Authors Apichat Asavamongkolkul, Department of Orthopaedic Surgery, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, 10700 ThailandSaranatra Waikakul, Department of Orthopaedic Surgery, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, 10700 Thailand Journal International OrthopaedicsOnline ISSN 1432-5195Print ISSN 0341-2695 (Source: International Orthopaedics)

Cervical chondroid chordoma in a standard dachshund: a case report

Acta Veterinaria Scandinavica — Fri, 21 Oct 2011 04:00:00 +0100

A ten-year-old male standard dachshund was presented with a history of neck pain and progressive gait disturbances. Following a neurological examination and diagnostic imaging, including CT, a neoplastic lesion involving the third and fourth cervical vertebrae was suspected. The lesion included an extradural mass on the right side of the spinal canal causing a local compression of the cervical cord. Surgery, using a modified dorsal laminectomy procedure, was performed in order to decompress the cervical spinal cord. Histopathological examination of the extradural mass indicated that the tumour was a chondroid chordoma. Following discharge, the quality of life for the dog was very good for a sustained period, but clinical signs recurred at 22 months. The dog was euthanased 25 months post-su...

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P63 does not regulate brachyury expression in human chordomas and osteosarcomas

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

(Source: Histopathology)

Current therapeutic options and novel molecular markers in skull base chordomas

Neurosurgical Review — Mon, 17 Oct 2011 16:03:22 +0100

We present a review of the current knowledge about skull base chordomas biology, therapeutic options and related clinical outcome. Content Type Journal ArticleCategory ReviewPages 1-14DOI 10.1007/s10143-011-0354-1Authors Filippo Gagliardi, Department of Neurosurgery, San Raffaele Scientific Institute, Vita-Salute University, Milan, ItalyNicola Boari, Department of Neurosurgery, San Raffaele Scientific Institute, Vita-Salute University, Milan, ItalyPaola Riva, Department of Genetics, University of Milan, Milan, ItalyPietro Mortini, Department of Neurosurgery, San Raffaele Scientific Institute, Vita-Salute University, Milan, Italy Journal Neurosurgical ReviewOnline ISSN 1437-2320Print ISSN 0344-5607 (Source: Neurosurgical Review)

Disseminated subarachnoid chordoma: long-term favorable follow-up of a pediatric patient

Pediatric Radiology — Sat, 08 Oct 2011 15:42:15 +0100

We describe a case of extraosseous chordoma disseminated in the subarachnoid space with favorable long-term follow-up. During work-up of headaches in a 13-year-old girl, MRI revealed multiple cystic subarachnoid masses in the posterior fossa and spinal canal. She underwent posterior fossa craniectomy and was found to have multicentric subarachnoid chordomas with positive CSF cytology. Six years after the operation and radiotherapy, the girl is without neurological deficits despite persistent multiple subarachnoid cystic masses. Content Type Journal ArticleCategory Case ReportPages 1-3DOI 10.1007/s00247-011-2266-1Authors Seth Anderson, Department of Radiology 3892 JPP, University of Iowa Hospitals and Clinics, 200 Hawkins Drive, Iowa City, IA 52242, USAYutaka Sato, Department of ...

Efficacy of Epidermal Growth Factor Receptor targeting in advanced chordoma: case report and literature review

BMC Cancer — Tue, 04 Oct 2011 04:00:00 +0100

Conclusions: We report the fourth case of advanced chordoma treated with an EGFR inhibitor. We also review the literature concerning the rationale and potential of EGFR targeting in chordoma. (Source: BMC Cancer)

Proton Beam Therapy in Skull Base Pathology

Otolaryngologic clinics of North America — Sat, 01 Oct 2011 04:00:00 +0100

The optimal treatment of chordomas is maximal safe resection followed by radiation therapy. Data have shown that the use of protons has increased the local control of chordomas. Because of their physical properties, proton therapy has a sharp decline at its distal range, thereby minimizing collateral damage. However, this choice of radiation therapy has been limited based on the availability of resources. Given the high cost of proton facilities and improved techniques for other forms of radiation therapy, this article evaluates whether proton therapy is still superior to other radiation techniques in the treatment of chordomas. (Source: Otolaryngologic clinics of North America)

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Multiple epidural lumbar chordomas without bone involvement in a 17-year-old female: a case report

The Spine Journal — Sat, 01 Oct 2011 04:00:00 +0100

Conclusions: Epidural chordomas appear to have a better prognosis due, at least in part, to the ability to more easily completely excise them. (Source: The Spine Journal)

Prospective assessment of patient morbidity from prone sacral positioning.

Journal of Neurosurgery.Spine — Fri, 30 Sep 2011 04:00:00 +0100

Conclusions A positioning protocol using head suspension on an open radiolucent frame facilitates oncological sacral surgery with reasonable patient morbidity. Morbid obesity and procedure times in excess of 10 hours are risk factors for positioning-related complications. To the authors' knowledge, this is the first report of surgical positioning morbidity in this patient population. PMID: 21962033 [PubMed - as supplied by publisher] (Source: Journal of Neurosurgery.Spine)

Side-Cutting Aspiration Device for Endoscopic and Microscopic Tumor Removal

Skull Base — Fri, 16 Sep 2011 04:00:00 +0100

Skull BaseDOI: 10.1055/s-0031-1287679ABSTRACTThe authors present a unique side-cutting instrument (NICO Myriad, Indianapolis, IN) with variable aspiration designed specifically for tumor resection. The study included retrospective review of data collected from 10/2009 to 01/2011. We detail the use of the Myriad in 31 patients with the following pathologies: meningioma (n = 16), chordoma (n = 3), schwannoma (n = 3), pituitary adenoma (n = 2), metastasis (n = 3), hemangioblastoma (n = 1), craniopharyngioma (n = 1), and nasopharyngeal tumors (n = 2). Surgical approaches included expanded endonasal approach (n = 19), endoscopic brain port (n = 3), supraorbital “eyebrow” craniotomy (n = 3), retrosigmoid suboccipital craniotomy (n = 3), pterion...

Presacral parachordoma causing intestinal obstruction in a patient with renal failure: Report of a case

Surgery Today — Thu, 15 Sep 2011 15:52:41 +0100

Abstract Parachordoma is usually lobulated and pseudoencapsulated, grows slowly, and is only locally invasive. It rarely occurs in the retroperitoneal area. Differential diagnoses include chordoma, extraskeletal myxoid chondrosarcoma, and subcutaneous sacrococcygeal myxopapillary ependymoma. We herein report our experience with a huge presacral parachordoma causing intestinal obstruction. A 48-year-old woman was a hemodialysis-dependent patient with end-stage renal disease. She had been diagnosed with uterine myomas several years prior. The patient presented with signs of intestinal obstruction. A presacral tumor with severe compression on the rectum resulting in rectal stricture was found. A resection of the presacral tumor was difficult owing to its large size and the di...

Durable complete response to chemotherapy in an infant with a clival chordoma

Pediatric Blood and Cancer — Thu, 15 Sep 2011 04:00:00 +0100

We report a 7‐month‐old infant with a clival chordoma who responded to combination chemotherapy consisting of cycles of vincristine/cyclophosphamide/doxorubicin alternating with etoposide/ifosfamide. She has been off chemotherapy for 2 years and is well at age 5. Pediatr Blood Cancer © 2011 Wiley‐Liss, Inc. (Source: Pediatric Blood and Cancer)

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In vitro characterization of cells derived from chordoma cell line U-CH1 following treatment with X-rays, heavy ions and chemotherapeutic drugs

Radiation Oncology — Wed, 14 Sep 2011 04:00:00 +0100

Conclusion: Our data provide the first comprehensive cellular characterization using cells of chordoma origin and furnish the biological basis for successful clinical results of chordoma treatment by heavy ions. (Source: Radiation Oncology)

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...

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 ...

The effects of chemotherapeutic agents on differentiated chordoma cells.

Journal of Neurosurgery.Spine — Fri, 09 Sep 2011 04:00:00 +0100

Conclusions The results suggest that chordoma cells may be treated using the differentiation method in a more effective way than when they are treated with chemotherapeutic agents alone. This new approach may be an alternative method to conventional therapies in the treatment of chordoma. PMID: 21905773 [PubMed - as supplied by publisher] (Source: Journal of Neurosurgery.Spine)

PET response and tumor stabilization under erlotinib and bevacizumab treatment of an intracranial lesion non-invasively diagnosed as likely chordoma.

Clinical Neuropathology — Thu, 01 Sep 2011 04:00:00 +0100

This report shows that erlotinib and bevacizumab in combination may completely quench the transport of the essential amino acid methionine to a treatment refractory intracranial tumor bearing radiological and clinical characteristics of a chordoma. Further studies are necessary to establish this strategy as a treatment option for this indication. PMID: 21955928 [PubMed - in process] (Source: Clinical Neuropathology)

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Chordoma of the thoracic spine in an 89-year-old

European Spine Journal — Thu, 25 Aug 2011 05:45:47 +0100

Conclusion Chordoma is a highly uncommon epidural neoplasm of the thoracic spine and is vanishingly rare in elderly patients. This report documents the oldest reported patient with thoracic spine chordoma at 89-year-old. Their locally aggressive behavior typically prompts consideration of aggressive surgical resection. When tailored to the individual patient, such procedures are feasible without excessive morbidity even in elderly patients. Content Type Journal ArticleCategory Case ReportPages 1-5DOI 10.1007/s00586-011-1980-6Authors Ricardo Fontes, Rush University Medical Center, 1725 West Harrison, Suite 1115, Chicago, IL 60612, USAJohn E. O’Toole, Rush University Medical Center, 1725 West Harrison, Suite 1115, Chicago, IL 60612, USA Journal European Spin...

Chordomas of the skull base and cervical spine: clinical outcomes associated with a multimodal surgical resection combined with proton-beam radiation in 40 patients

Urosurgical Review — Tue, 23 Aug 2011 15:52:07 +0100

Abstract Previous studies of chordoma have focused on either surgery, radiotherapy, or particular tumor locations. This paper reviewed the outcomes of surgery and proton radiotherapy with various tumor locations. Between 2001 and 2008, 40 patients with chordomas of the skull base and cervical spine had surgery at our hospital. Most patients received proton therapy. Their clinical course was reviewed. Age, sex, tumor location, timing of surgery, extent of resection, and chondroid appearance were evaluated in regard to the progression-free survival (PFS) and overall survival (OS). The primary surgery (PS) group was analyzed independently. The extensive resection rate was 42.5%. Permanent neurological morbidity was seen in 3.8%. Radiotherapy was performed in 75% and the mean ...

Spontaneous Debulking of Middle Fossa Chordoma Extension after Transnasal Petroclival Biopsy – Report of a Case

min - Minimally Invasive Neurosurgery — Mon, 22 Aug 2011 23:00:00 +0100

Minim Invasive Neurosurg 2011; 54: 135-137DOI: 10.1055/s-0031-1283128Abstract[...]Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals:Table of contents | Abstract | Full text (Source: min - Minimally Invasive Neurosurgery)

Current comprehensive management of cranial base chordomas: 10-year meta-analysis of observational studies.

Journal of Neurosurgery — Thu, 04 Aug 2011 23:00:00 +0100

Conclusions Patients with complete resection of cranial base chordomas have a prolonged 5-year PFS and OS. Adjuvant proton-beam, carbon ion, and modern fractionated photon radiation therapy techniques offered a similar rate of PFS and OS at 5 years. PMID: 21819197 [PubMed - as supplied by publisher] (Source: Journal of Neurosurgery)

Percutaneous Intratumoral Injection With Pingyangmycin Lipiodol Emulsion for the Treatment of Recurrent Sacrococcygeal Chordomas

Journal of Vascular and Interventional Radiology : JVIR — Fri, 29 Jul 2011 17:55:14 +0100

This study describes fluoroscopy-guided percutaneous intratumoral injection therapy (PIIT) with a pingyangmycin lipiodol emulsion in the management of recurrent sacrococcygeal chordomas after surgical excision. Seven patients underwent a total of 22 treatment sessions (3–4 sessions per patient); treatment responses were evaluated clinically, and lesion size was determined using computed tomography (CT). Over 10–26 months of follow-up, tumor sizes and visual analogue scale (VAS) scores of all patients were decreased. No patients had complications during the follow-up period. Preliminary results showed that PIIT with pingyangmycin lipiodol emulsion under fluoroscopic guidance is effective and safe and may be considered as a treatment option. (Source: Journal of Vascular and Interventiona...

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A robust algorithm of intensity modulated proton therapy for critical tissue sparing and target coverage.

Physics in Medicine and Biology — Thu, 21 Jul 2011 14:03:30 +0100

In this study, the latter objective was achieved by a novel algorithm in which we suppressed pencil beams with high risk to deliver undesired doses to organs at risk under conditions where range and setup uncertainties occur. We defined the risk index that quantifies the likelihood of each pencil beam delivering high doses to organs at risk, and introduced it into the objective function of dose optimizations. In order to test the algorithm's performance, this method was applied to an RTOG benchmark phantom geometry and to a cervical chordoma case. These simulations demonstrated that our method provides IMPT plans that are more robust against range and setup errors compared to conventional IMPT plans. Compared to the conventional IMPT plan, the optimization time for the robust plan increase...

Skull Base Chordomas

Otolaryngologic clinics of North America — Thu, 21 Jul 2011 04:00:00 +0100

Skull base chordomas are rare midline malignancies of clival origin that represent one of the most challenging skull base tumors to treat, given their location, invasiveness, potential extension around vital neurovascular structures, and high recurrence rate. Total tumor resection is the mainstay of treatment. The combination of surgery and postoperative irradiation appears to provide the best outcome. (Source: Otolaryngologic clinics of North America)

Coccydynia secondary to a large pelvic tumor of anorectal origin

The Spine Journal — Thu, 07 Jul 2011 23:00:00 +0100

This 57-year-old woman described symptoms typical of idiopathic coccydynia , which continued to worsen over a 2-year period despite joint manipulation, steroid injection, sacrococcygeal nerve blocks, fentanyl patches, and pregabalin. She subsequently presented with a presumed pilonidal abscess. Examination under anesthetic revealed fibrous ulceration in the natal cleft with a firm mass on digital rectal examination extending into the left ischiorectal fossa. There were no previous symptoms of anorectal pathology, and a magnetic resonance imaging lumbar spine had been normal 1-year earlier. Postoperative magnetic resonance imaging of the pelvis revealed a large mass suspicious for sarcoma or chordoma (). However, biopsies from the perianal lesion demonstrated mucinous adenocarcinoma, with ...

Inadvertent insertion of nasogastric tube into the brain stem and spinal cord after endoscopic skull base surgery

American Journal of Otolaryngology — Wed, 29 Jun 2011 04:00:00 +0100

We report a case of inadvertent placement of small-bore feeding tube into the brain stem and spinal cord in a patient with a history of previous endoscopic transnasal resection of clival chordoma. We discuss the management of this complication and the strategies that have been developed to avoid this complication in the future. (Source: American Journal of Otolaryngology)

Generation of chordoma cell line JHC7 and the identification of Brachyury as a novel molecular target.

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

Conclusions This report represents the first xenograft model of a sacral chordoma line described in the literature and the first cell line established with stable Brachyury expression. The authors propose that Brachyury is an attractive therapeutic target in chordoma and that JHC7 will serve as a clinically relevant model for the study of this disease. PMID: 21699479 [PubMed - as supplied by publisher] (Source: Journal of Neurosurgery)

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Clinical features distinguish childhood chordoma associated with tuberous sclerosis complex (TSC) from chordoma in the general paediatric population

Journal of Medical Genetics — Tue, 21 Jun 2011 23:00:00 +0100

Conclusions These results strengthen the association between paediatric chordoma and TSC. Future clinical and molecular studies documenting the magnitude and clinical spectrum of the joint occurrence of these two diseases should provide the basis for delineating the biological relationship between them. (Source: Journal of Medical Genetics)

Re-irradiation with scanned charged particle beams in recurrent tumours of the head and neck: Acute toxicity and feasibility

Radiotherapy and Oncology — Mon, 06 Jun 2011 04:00:00 +0100

Conclusion: Initial experience of re-irradiation with scanned particle beams in recurrent tumours of the head and neck seems feasible and encouraging. Further follow-up is needed to investigate potential late effects. (Source: Radiotherapy and Oncology)

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)

Re-irradiation with scanned charged particle beams in recurrent tumours of the head and neck: Acute toxicity and feasibility.

Tue, 31 May 2011 23:00:00 +0100

CONCLUSION: Initial experience of re-irradiation with scanned particle beams in recurrent tumours of the head and neck seems feasible and encouraging. Further follow-up is needed to investigate potential late effects. PMID: 21640421 [PubMed - as supplied by publisher] (Source: Radiotherapy and Oncology : journal of the European Society for Therapeutic Radiology and Oncology)

Effective Use of Thalidomide in the Treatment of Recurrent Metastatic Chordoma [DIAGNOSIS IN ONCOLOGY]

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

(Source: Journal of Clinical Oncology)

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Systemic Therapy Options for Unresectable and Metastatic Chordomas

Current Oncology Reports — Mon, 16 May 2011 08:42:22 +0100

Abstract Chordoma is an exceedingly rare tumor, marked by a slow growth rate. Surgery is the treatment of choice, but the most frequent sites of origin (spine and skull base) make treatment of primary disease challenging. Local relapses affect more than 50% of cases, with a minority of patients being cured by further surgery. Furthermore, metastases occur in at least 20% of patients. For residual or recurrent disease, high-dose radiation therapy may be indicated. Radiation therapy is currently the preferred local treatment when surgery is problematic, exploiting most recent techniques, including proton beams and carbon ions. However, systemic therapy is needed in patients non-amenable to surgery and/or radiation therapy. We reviewed systemic treatments currently available,...

A Rare Case of Jugular Foramen Chordoma With an Unusual Extension [Clinical Note]

Archives of Otolaryngology — Sun, 15 May 2011 23:00:00 +0100

(Source: Archives of Otolaryngology)

Spot-Scanning-Based Proton Therapy for Extracranial Chordoma

International Journal of Radiation Oncology * Biology * Physics — Mon, 18 Apr 2011 04:00:00 +0100

Conclusion: Spot-scanning-based PT at PSI delivered subsequently to function-preserving surgery for tumor debulking, decompression of spinal cord, or biopsy only is safe and highly effective in patients with ECC without major surgical instrumentation even in view of large, unresectable disease. (Source: International Journal of Radiation Oncology * Biology * Physics)

Sphenoid Masses in Children: Radiologic Differential Diagnosis with Pathologic Correlation [REVIEW ARTICLE]

American Journal of Neuroradiology — Mon, 11 Apr 2011 23:00:00 +0100

SUMMARY: Childhood central skull base masses are rare, often difficult to diagnose, and have overlapping imaging findings. In this review, we provide an overview of the epidemiology, clinical findings, and management of pediatric sphenoid bone and sphenoid sinus masses with an emphasis on imaging findings that may help to differentiate lesions. Radiologic-pathologic correlation is provided. Finally, an imaging-based algorithm is presented as a guide to help radiologists narrow their differential diagnoses. Some of the entities discussed are virtually unique to the pediatric population; others occur rarely in this age group but should be considered in the appropriate clinical setting. Entities included in the discussion are grouped into 2 categories: those that cause nonaggressive osseous r...

Extensive lumbar chordoma and unique reconstructive approach

European Spine Journal — Sat, 09 Apr 2011 15:49:08 +0100

We describe a case of an extensive lumbar chordoma, and demonstrate the possibility of performing a staged anterior and posterior total tumour resection with planned transgression in conjunction with an expandable cage, vascularised rib, fibula and latissimus dorsi grafts for the treatment and reconstruction of extensive spinal disease with this condition. Content Type Journal ArticlePages 1-7DOI 10.1007/s00586-011-1785-7Authors Praveenan Sivabalan, University of New South Wales, 18/8-12 Ascot St, Kensington, NSW 2033, AustraliaJane Li, University of New South Wales, 17 Biralee Crescent, Beacon Hill, NSW 2100, AustraliaRalph J. Mobbs, Department of Neurosurgery, Prince of Wales Spine Unit, Sydney Spine Clinic, Prince of Wales Hospital, Suite 3, Level 7, Randwick, NSW 2031, Austral...

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Extraosseous chordoma of nasopharynx in paediatric age.

Acta Otorrinolaringologica Espanola — Thu, 31 Mar 2011 23:00:00 +0100

We present a case of primarily extraosseous chordoma in paediatric age, involving the nasopharynx, to improve preoperative diagnosis and postoperative management of this rare tumour. PMID: 21459353 [PubMed - as supplied by publisher] (Source: Acta Otorrinolaringologica Espanola)

Use of a side-cutting aspiration device for resection of tumors during endoscopic endonasal approaches.

Neurosurgical Focus — Thu, 31 Mar 2011 23:00:00 +0100

Conclusions The side-cutting tissue resector is a safe, easy to use, and effective tool for internal debulking and extracapsular dissection of nonvascularized tumors that are too firm for bimanual suction or blunt ring curette dissection. It is particularly useful when working through a deep and narrow corridor such as is encountered in endoscopic endonasal skull base surgery. PMID: 21456924 [PubMed - in process] (Source: Neurosurgical Focus)

Degenerative Pannus Mimicking Clival Chordoma Resected via an Endoscopic Transnasal Approach

Skull Base — Tue, 29 Mar 2011 23:00:00 +0100

Skull BaseDOI: 10.1055/s-0031-1275243ABSTRACTLesions of the lower clivus represent a technically challenging subset of skull base disease that requires careful treatment. A 75-year-old woman with tongue atrophy was referred for resection of a presumed clival chordoma. The lesion was resected via an endoscopic transnasal transclival approach with no complications. Pathology revealed only chronic inflammatory tissue consistent with a degenerative pannus. Degenerative pannus should be included in the differential diagnosis of lower clival extradural lesions. The endoscopic transnasal transclival corridor should be considered for resection of such lesions as an alternative to larger, more morbid, traditional skull base approaches.[...]© Thieme Medical PublishersArticle in Thieme eJournals:Tab...

Feasibility of Proton Beam Therapy for Chordoma and Chondrosarcoma of the Skull Base

Skull Base — Fri, 25 Mar 2011 00:00:00 +0100

Skull BaseDOI: 10.1055/s-0031-1275636ABSTRACTWe explored the general feasibility of proton beam therapy for chordoma and chondrosarcoma of the skull base. Clinical records and treatment-planning data of patients with the pathological diagnosis of chordoma or chondrosarcoma were examined. Proton beam therapy was administered for gross tumor mass as well as microscopic residual disease after surgery. The prescribed dose was determined to maximize the coverage of the target and to not exceed predefined constraints for the organs at risk. Eight cases of chordoma and eight cases of chondrosarcoma were enrolled. The median tumor volume was 40 cm3 (range, 7 to 546 cm3). The prescribed dose ranged from 50 to 70 Gy (relative biological effectiveness [RBE]), with a median of 63 Gy RBE. The median fo...

On “Clivus Chordoma: Is It Enough to Image the Primary Site?” (Skull Base 2010;20:111-113)

Skull Base — Mon, 21 Mar 2011 00:00:00 +0100

Skull BaseDOI: 10.1055/s-0031-1275261© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents | Full text (Source: Skull Base)

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On “Clivus Chordoma: Is It Enough to Image the Primary Site?” (Skull Base 2010;20:111–113)

Skull Base — Mon, 21 Mar 2011 00:00:00 +0100

Skull BaseDOI: 10.1055/s-0031-1275261© Thieme Medical PublishersArticle in Thieme eJournals:Table of contents | Full text (Source: Skull Base)

Positional Correction Data Prove the Absolute Necessity for Image Guidance in High Dose Radiotherapy for Spinal Chordoma

Clinical Oncology — Tue, 15 Mar 2011 18:27:11 +0100

Aims: Patients with spinal chordoma require high dose postoperative radiotherapy, and appear to benefit from image guidance. Patients: We reviewed a case of T12 chordoma treated on the TomoTherapy HiArtTM system using daily imaging and positional correction (DIPC), and IMRT to deliver 70 Gy and 39 fractions. Positional corrections were noted. The effects of four imaging strategies were evaluated: no imaging, imaging and correction for the first 3 days with subsequent mean correction (SMC), imaging and correction for the first 5 days with SMC, and finally DIPC. In-house software (SHERRI) was used to evaluate changes in the DVH, and likely changes in tumour control probability (TCP) and toxicity using the equivalent uniform dose (EUD) concept. (Source: Clinical Oncology)

Chordoma and Chondrosarcoma of the Skull Base and Spine: Small Post-operative Tumour Volume may still be Curable after High Dose Photon Radiotherapy

Clinical Oncology — Tue, 15 Mar 2011 18:27:07 +0100

Aims: High dose radiotherapy (RT) following surgical debulking is the treatment of choice for chordomas and chondrosarcomas. While many patients are now eligible for proton RT abroad, those with spinal tumours requiring metal reconstruction implants are probably better treated with photons. Can such patients still be cured if there is any residual postoperative tumour? (Source: Clinical Oncology)

Endocrinological outcome after pituitary transposition (hypophysopexy) and adjuvant radiotherapy for tumors involving the cavernous sinus.

Journal of Neurosurgery — Fri, 11 Mar 2011 00:00:00 +0100

Conclusions The increased distance between the normal pituitary gland and the residual tumor facilitates treatment of the tumor with radiosurgery or radiotherapy and effectively reduces the incidence of radiation injury to the normal pituitary gland when compared with historical controls. PMID: 21395389 [PubMed - as supplied by publisher] (Source: Journal of Neurosurgery)

Extra-axial soft tissue chordoma of wrist.

Pathology, Research and Practice — Thu, 10 Mar 2011 00:00:00 +0100

We report a case of extra-axial soft tissue chordoma of the right wrist in an 87-year-old man. The tumor was large, and the cut surface showed multinodular and myxoid appearance. Microscopically, nests of epithelioid and spindle cells were observed in the myxoid matrix. Vacuolated cells were also observed. The tumor cells were diffusely positive for brachyury and cytokeratin 19 on immunohistochemistry, suggesting that the tumor was extra-axial soft tissue chordoma. Extra-axial chordoma is the same entity as chordoma periphericum (parachordoma), as proposed by Laskowski and Dabska. PMID: 21397407 [PubMed - as supplied by publisher] (Source: Pathology, Research and Practice)

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The biological basis for modern treatment of chordoma

Journal of Neuro-Oncology — Tue, 08 Mar 2011 06:44:49 +0100

Abstract Chordomas are rare malignant tumors arising in bone of the spheno-occiput, sacrum, and vertebral column which can cause neurological deficit. Current management of chordoma involves safe resection followed by radiation therapy. However, surgical resection is often subtotal and chordoma often recurs despite optimal therapy. Despite years of effort, effective adjuvant therapy for denovo, recurrent and metastatic chordoma are absent and 5-year survival is at best 65%. While no chemotherapeutic agent has been demonstrated to be effective against chordoma in vivo, a greater understanding of the genetics and molecular biology of chordoma is opening up avenues of investigation towards the rational development of targeted therapies. Although enthusiasm for the use of alre...

Encouraging Early Clinical Outcomes With Helical Tomotherapy–Based Image-Guided Intensity-Modulated Radiation Therapy for Residual, Recurrent, and/or Progressive Benign/Low-Grade Intracranial Tumors: A Comprehensive Evaluation

International Journal of Radiation Oncology * Biology * Physics — Wed, 23 Feb 2011 05:00:00 +0100

Conclusions: Careful selection of radiotherapy technique is warranted for benign/low-grade brain tumors to achieve durable local control with minimum long-term morbidity. Large or complex-shaped tumors benefit most from IMRT. Our early clinical experience of HT-based IMRT for brain tumors has been encouraging. (Source: International Journal of Radiation Oncology * Biology * Physics)

Differentiating Benign Notochordal Cell Tumors From Chordomas: Radiographic Features on MRI, CT, and Tomography

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

CONCLUSION. Radiologic studies may allow distinction of BNCTs from chordomas. (Source: American Journal of Roentgenology)

Chordoid meningioma of the third ventricle: a case report and review of the literature.

Clinical Neuropathology — Mon, 21 Feb 2011 00:00:33 +0100

We present a case of a chordoid meningioma that presented in the third ventricle of a 63-year-old female. This represents only the second documented case of a third ventricular chordoid meningioma and the first case in an adult. The distinction from other chordoid neoplasms can be challenging, particularly chordoid gliomas, which classically occur in this location. Herein, we compare and contrast chordoid meningioma with chordoid glioma and provide a review of the relevant literature. PMID: 21329615 [PubMed - as supplied by publisher] (Source: Clinical Neuropathology)

Pediatric Metastatic Sacrococcygeal Chordoma Treated with Surgery

European Journal of Pediatric Surgery — Mon, 21 Feb 2011 00:00:00 +0100

Eur J Pediatr SurgDOI: 10.1055/s-0031-1271635© Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals:Table of contents | Full text (Source: European Journal of Pediatric Surgery)

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Assessment of Early Toxicity and Response in Patients Treated With Proton and Carbon Ion Therapy at the Heidelberg Ion Therapy Center Using the Raster Scanning Technique

International Journal of Radiation Oncology * Biology * Physics — Mon, 07 Feb 2011 05:00:00 +0100

Conclusions: Side effects related to particle treatment were rare, and the overall tolerability of the treatment was shown. The initial response was promising. The data have confirmed the safe delivery of carbon ions and protons at the newly opened Heidelberg facility. (Source: International Journal of Radiation Oncology * Biology * Physics)

The Transnasal Transclival Approach for Clivus Chordoma

min - Minimally Invasive Neurosurgery — Mon, 07 Feb 2011 00:00:00 +0100

We present our experience using a standardized transnasal transclival approach (TTA) for endoscopic removal of chordomas of the clivus.[...]© Georg Thieme Verlag KG Stuttgart · New YorkArticle in Thieme eJournals:Table of contents | Abstract | Full text (Source: min - Minimally Invasive Neurosurgery)

Preoperative grading scale to predict survival in patients undergoing resection of malignant primary osseous spinal neoplasms

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

Abstract: Background context: Large population-based studies of malignant primary osseous spinal neoplasms are lacking and are necessary to have sufficient statistical power to determine if various patient-related factors are in fact significant indicators of prognosis.Purpose: Using a 30-year US national cancer registry (Surveillance, Epidemiology, and End Results [SEER]), we introduce a preoperative grading scale that is associated with survival in patients undergoing surgical resection for malignant primary osseous spinal neoplasms.Study design: Large-scale retrospective study.Patient sample: SEER registry.Outcome measure: Survival.Methods: The SEER registry (1973–2003) was queried to identify adult patients undergoing surgical resection of histologically confirmed primary spinal chor...

Chondroid chordoma of the skull base: immunohistochemical and ultrastructural study of two cases with special reference to microtubules within rough-surfaced endoplasmic reticulum

Medical Molecular Morphology — Wed, 26 Jan 2011 02:57:57 +0100

Abstract Two cases of skull base chordoma (case 1, a 57-yearold woman; case 2, a 69-year-old woman) were investigated immunohistochemically and ultrastructurally. The tumors showed histopathological features typical of chondroid chordoma and contained both classical chordomatous and hyaline cartilaginous components. Tumor cells were immunoreactive for cytokeratin, vimentin, and S-100 protein, but negative for microtubule-associated protein 2 and class III beta-tubulin (tub-B3). Tumor cells of case 2 were immunoreactive for tauprotein and class II beta-tubulin (tub-B2), whereas those of case 1 were negative. Ultrastructurally, tumor cells in both cases showed the presence of abundant glycogen granules, well-developed intracellular organelles, and desmosome-like junctions. I...

Pulsed laser-induced liquid jet for skull base tumor removal with vascular preservation through the transsphenoidal approach: a clinical investigation

Acta Neurochirurgica — Thu, 13 Jan 2011 07:04:33 +0100

Conclusion Although comparison between conventional surgical instruments is mandatory in the future, the present study suggests that the LILJ system can achieve safe and optimum removal of complex skull base tumor. Potential application for minimally invasive endoscopic system, as well as potentials for changing the design of the catheter in according to preference of surgeon with low cost, may give advantages over conventional surgical instruments. Content Type Journal ArticlePages 1-8DOI 10.1007/s00701-010-0925-xAuthors Yoshikazu Ogawa, Department of Neurosurgery, Kohnan Hospital, 4-20-1 Nagamachiminami, Taihaku-ku, Sendai, Miyagi 982-8523, JapanAtsuhiro Nakagawa, Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi JapanKazuy...

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The treatment outcome depending on the extent of resection in skull base and spinal chordomas

Acta Neurochirurgica — Wed, 05 Jan 2011 06:45:11 +0100

Conclusions Adjunctive radiotherapy/radiosurgery following subtotal resection showed comparable local control and survival to wide resection. SRS offered superior local tumour control compared to EBRT. Content Type Journal ArticlePages 1-8DOI 10.1007/s00701-010-0928-7Authors Ahmed Shawky Eid, Department of Orthopedic Surgery, Ain Shams University, Cairo, EgyptUng-Kyu Chang, Department of Neurosurgery, Korea Cancer Center Hospital, 215-4, Gongneung-dong, Nowon-gu, Seoul, 139-706 South KoreaSoo-Yong Lee, Department of Orthopedic Surgery, Korea Cancer Center Hospital, Seoul, South KoreaDae-Geun Jeon, Department of Orthopedic Surgery, Korea Cancer Center Hospital, Seoul, South Korea Journal Acta NeurochirurgicaOnline ISSN 0942-0940Print ISSN 0001-6268 (Source: Act...

Unveiling the molecular pathogenesis of chordoma: a new paradigm for molecular targeting of rare cancers

The Journal of Pathology — Wed, 05 Jan 2011 00:00:00 +0100

AbstractChordoma represent orphan sarcoma subtypes in which systemic treatments have proved so far ineffective. Two original papers in The Journal of Pathology have recently elucidated important steps of its molecular oncogenesis. The demonstration of gains of copy numbers of both T (brachyury homologue) and EGFR (epidermal growth factor receptor) genes in chordoma, as well as of their role in promoting cell proliferation, offers a strong preclinical rationale for exploring the inhibition of their products as a credible therapeutic strategy. When dealing with rare cancers, clinical trials based on a frequency‐based statistical methodology are hardly feasible. To override the limitations of rarity, the clinical community is suggesting the adoption of a Bayesian methodology. The success of...

Chordoma.

Ear, Nose and Throat Journal — Sat, 01 Jan 2011 00:00:00 +0100

Authors: Thompson LD PMID: 21229504 [PubMed - in process] (Source: Ear, Nose and Throat Journal)

Transfacial approach, pedicled rhinotomy for a clival chordoma: a technical report.

Turkish Neurosurgery — Sat, 01 Jan 2011 00:00:00 +0100

We present a case operated on with the pedicled transnasal and transfacial approach. The case presented with neurological deficits as cranial nerve palsy and findings of brainstem compression. The lesion was removed without any neurological deficit. Her deficits related to brainstem compression regressed after surgery. In our case, a large exposure was achieved through a lateral nasal incision in order to excise the tumor totally with acceptable cosmetic results, and a successful outcome was observed during the postoperative follow-up period with the surgical procedure applied. PMID: 21294097 [PubMed - in process] (Source: Turkish Neurosurgery)

Molecular Characterization of Putative Chordoma Cell Lines

Sarcoma — Thu, 30 Dec 2010 22:45:34 +0100

Immortal tumor cell lines are an important model system for cancer research, however, misidentification and cross-contamination of cell lines are a common problem. Seven chordoma cell lines are reported in the literature, but none has been characterized in detail. We analyzed gene expression patterns and genomic copy number variations in five putative chordoma cell lines (U-CH1, CCL3, CCL4, GB60, and CM319). We also created a new chordoma cell line, U-CH2, and provided genotypes for cell lines for identity confirmation. Our analyses revealed that CCL3, CCL4, and GB60 are not chordoma cell lines, and that CM319 is a cancer cell line possibly derived from chordoma, but lacking expression of key chordoma biomarkers. U-CH1 and U-CH2 both have gene expression profiles, copy number aberrations, ...

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Polyvinylpyrrolidone storage disease presenting as pathologic fracture and anemia: Report of a case with imprint cytology

Diagnostic Cytopathology — Thu, 30 Dec 2010 00:00:00 +0100

AbstractPolyvinylpyrrolidone (PVP) storage disease can be caused by local injection and systemic parenteral administration of PVP‐containing solutions. PVP has been used as plasma expander, a retardant in certain medicines, components of food additive, and hair care products. High‐molecular‐weight PVP polymers are prevented from renal excretion and are retained in the reticuloendothelial system. The clinical manifestations include skin lesions and hematologic and orthopedic complications because of bone marrow failure and bony destruction with infiltration of PVP storage histiocytes. Herein, we report a 65‐year‐old female patient with PVP storage disease presenting as femoral fracture and anemia. In our case, some gelatinous material was noted atthe fracture site, and the initial...

Unusual localization of an unusual tumor: calcifying pseudoneoplasm of the foramen magnum.

Clinical Neuropathology — Sun, 26 Dec 2010 16:12:06 +0100

Conclusion: We report, to our knowledge, the second case of a calcifying pseudoneoplasm of the foramen magnum. We emphasize that these tumors are pathologically benign and do not require aggressive surgical treatment and suggest that asymptomatic cases can be followed radiologically. PMID: 21176714 [PubMed - in process] (Source: Clinical Neuropathology)

Survival of patients with malignant primary osseous spinal neoplasms: results from the Surveillance, Epidemiology, and End Results (SEER) database from 1973 to 2003.

Journal of Neurosurgery.Spine — Fri, 24 Dec 2010 00:00:00 +0100

Conclusions In this analysis of a 30-year, US population-based cancer registry (SEER), the authors provide nationally representative prognosis and survival data for patients with malignant primary spinal osseous neoplasms. Overall patient survival has improved for isolated spine tumors with advancements in care over the past 4 decades. These results may be helpful in providing historical controls for understanding the efficacy of new treatment paradigms, patient education, and guiding level of aggressiveness in treatment strategies. PMID: 21184634 [PubMed - as supplied by publisher] (Source: Journal of Neurosurgery.Spine)

Expression of MDR1, HIF-1alpha and MRP1 in sacral chordoma and chordoma cell line CM-319

Journal of Experimental and Clinical Cancer Research — Wed, 08 Dec 2010 00:00:00 +0100

Conclusion: Expression of HIF-1alpha and MRP1 was observed in most chordoma specimen and CM-319 cell line; expression of HIF-1alpha correlated with MRP1. HIF-1alpha and MRP1 may play a role in the multidrug resistance of chordoma to chemotherapy. (Source: Journal of Experimental and Clinical Cancer Research)

Carbon ion radiotherapy performed as re-irradiation using active beam delivery in patients with tumors of the brain, skull base and sacral region

Radiotherapy and Oncology — Mon, 29 Nov 2010 00:00:00 +0100

Conclusion: Due to the physical characteristics particle therapy offers a new treatment modality in cases with tumor recurrences. With carbon ions, the additional biological benefits may be exploited for long-term tumor control. Further evaluation in a larger patients’ cohort will be performed in the future. (Source: Radiotherapy and Oncology)

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Proteomic profiling of chordoma

Journal of Surgical Oncology — Wed, 24 Nov 2010 01:57:10 +0100

(Source: Journal of Surgical Oncology)

Sphenoid Masses in Children: Radiologic Differential Diagnosis with Pathologic Correlation [REVIEW ARTICLE]

American Journal of Neuroradiology — Wed, 24 Nov 2010 00:00:00 +0100

Specificity of brachyury in the distinction of chordoma from clear cell renal cell carcinoma and germ cell tumors: a study of 305 cases

Modern Pathology AOP — Fri, 19 Nov 2010 00:00:00 +0100

Authors: Ankur R Sangoi, Jason Karamchandani, Brooke Lane, John P Higgins, Robert V Rouse, James D Brooks & Jesse K McKenney (Source: Modern Pathology AOP)

Cervical chordoma managed with multidisciplinary surgical approach

ANZ Journal of Surgery — Wed, 17 Nov 2010 00:00:00 +0100

Conclusion: An interdisciplinary approach should be used in the management of upper cervical chordomas to facilitate tumour resection and reduce the potential for recurrence. (Source: ANZ Journal of Surgery)

Fine‐needle aspiration cytology with histological correlation of chordoma metastatic to the lung: A diagnostic dilemma

Diagnostic Cytopathology — Tue, 09 Nov 2010 00:00:00 +0100

AbstractChordoma is an uncommon tumor initially believed to be benign due to the rarity of its metastasis. Cytological, morphological, and immunohistochemical features of chordoma, relating to its origin from notochordal remnants, allows for its accurate diagnosis. A 75‐year‐old man with a known history of tuberculosis (TB) presented with shortness of breath and a dry cough. A chest X‐ray demonstrated a diffuse, infiltrative miliary pattern in both lungs. Bronchial washings submitted for culture and cytological examination did not identify any tuberculous bacilli. Fine needle aspiration cytology (FNAC) showed focal areas of myxoid regions with small, round, uniform mononuclear cells. There was a documented past history of chordoma arising from the L2 vertebrae. Because of the rarity ...

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Intensity modulated radiotherapy in the management of sacral chordoma in primary versus recurrent disease

Radiotherapy and Oncology — Tue, 09 Nov 2010 00:00:00 +0100

We report on 34 patients with histologically proven sacral chordoma. Seventeen patients were treated at time of initial diagnosis with post-operative IMRT (n=13) or with IMRT alone (n=4). Seventeen patients were treated in recurrent disease after surgery (n=11) or with radiotherapy alone (n=6). Median total dose to the boost volume (PTV2) was 66Gy (range, 72–54) with 2Gy per fraction using an integrated boost concept. Median dose to target volume (PTV1) was 54Gy in 1.8Gy.Results: Local control was 35% (12/34) and overall survival 74% (25/34) after a median follow-up of 4.5years. Actuarial local control was 79%, 55% and 27% after 1, 2 and 5years, respectively. Local control was significantly higher in patients treated for primary tumors (p60Gy (p (Source: Radiotherapy and Oncology)

Malignant sarcoma of the pelvic bones

Cancer — Mon, 08 Nov 2010 00:00:00 +0100

CONCLUSIONS:This is an analysis of patients with pelvic sarcomas derived from a population‐based registry. Survival and prognostics vary with histopathological diagnoses. Although surgical resection was associated with superior outcomes for osteosarcoma and chondrosarcoma, there was no significant difference in outcomes of patients with Ewing sarcoma treated with surgery and/or radiotherapy. Cancer 2010. © 2010 American Cancer Society. (Source: Cancer)

Loss of SMARCB1/INI1 expression in poorly differentiated chordomas

Acta Neuropathologica — Sat, 06 Nov 2010 20:18:02 +0100

Abstract Chordomas are malignant neoplasms that typically arise in the axial spine and primarily affect adults. When chordomas arise in pediatric patients they are more likely to display unusual histological features and aggressive behavior. We noted the absence of SMARCB1/INI1 expression by immunohistochemistry in an index case of poorly differentiated chordoma of the sacrum, leading us to further examine SMARCB1/INI1 expression as well as that of brachyury, a highly specific marker of notochordal differentiation, in 3 additional poorly differentiated chordomas of the clivus, 10 typical chordomas, and 8 atypical teratoid/rhabdoid tumors (AT/RTs). All 4 poorly differentiated chordomas and all AT/RTs lacked nuclear expression of SMARCB1/INI1, while the 10 typical chordomas ...

Randomised trial of proton vs. carbon ion radiation therapy in patients with chordoma of the skull base, clinical phase III study (HIT-1-Study)

BMC Cancer — Fri, 05 Nov 2010 00:00:00 +0100

DiscussionUp until now it was impossible to compare two different particle therapies, i.e. protons and carbon ions directly at the same facility. The aim of this study is to find out, whether the biological advantages of carbon ion therapy can also be clinically confirmed and translated into the better local control rates in the treatment of skull base chordomas. (Source: BMC Cancer)

The role of chemotherapy in pediatric clival chordomas

Journal of Neuro-Oncology — Thu, 04 Nov 2010 17:49:18 +0100

Abstract The purpose of this retrospective study was to review the role of chemotherapy in the treatment, management and outcome of children diagnosed with clival chordomas. The medical records of six pediatric chordoma patients diagnosed at Childrens Hospital Los Angeles between 1995 and 2005 were reviewed. Of the six patients reviewed, all underwent an initial surgical resection. Following resection, three received a combination of chemotherapy and radiation therapy, two received chemotherapy alone and one patient refused both forms of therapy; this patient expired of progressive tumor. One patient developed acute monoblastic leukemia (M5a subtype) and died of intracranial hemorrhage during induction chemotherapy, 39 months after initial diagnosis. MRI of brain and ...

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Intensity modulated radiotherapy in the management of sacral chordoma in primary versus recurrent disease.

Thu, 04 Nov 2010 00:00:00 +0100

CONCLUSION: These data demonstrate that local control after IMRT is higher in patients treated for primary tumors and using higher radiation doses. Therefore, we recommend radiotherapy as part of initial treatment in sacral chordoma. PMID: 21056488 [PubMed - as supplied by publisher] (Source: Radiotherapy and Oncology : journal of the European Society for Therapeutic Radiology and Oncology)

Sacral and presacral lesions: Cytopathologic analysis and clinical correlates

Diagnostic Cytopathology — Tue, 02 Nov 2010 00:00:00 +0100

AbstractAlthough percutaneous biopsies of the spine at thoracolumbar levels have proven value, the benefit in the sacrococcygeal region has not been presented thoroughly in the literature. This region has unique anatomic and oncologic properties.The cytopathologic files at The Johns Hopkins Hospital over a 20‐year‐period were searched. Imaging studies and medical records were also reviewed.Ninety‐one cases of sacral/presacral FNA were identified. There were 46 males and 45 females; 62% had a prior history of malignancy. The most common presentation was lower back pain (44%). Imaging revealed lytic and/or soft tissue lesions masses from 1 to 12 cm (mean = 5.0 cm). Of the 71 (78%) diagnostic cases, 19 (27%) were nonneoplastic, 2 (3%) were suspicious for neoplasm, while 50 (70%) cases w...

[Externalization of a soft tissue chordoma. Case report.]

Cirugia y Cirujanos — Mon, 01 Nov 2010 00:00:00 +0100

Conclusions: Theoretically, notochord in the vertebral bodies is not completely degenerative and the remaining tissue persists, producing chordomas. The most common symptom is local pain. Diagnosis is made by imaging: x-ray of lumbosacral spine, computed tomography and magnetic resonance imaging. The treatment of choice is wide surgical resection. Diagnosis of chordoma is rare but should be kept in mind in patients with pain in lower back or coccyx and, even more so, if accompanied by a mass with years of evolution. PMID: 21214993 [PubMed - as supplied by publisher] (Source: Cirugia y Cirujanos)

The Role of Epidermal Growth Factor Receptor in Chordoma Pathogenesis: a Potential Therapeutic Target

The Journal of Pathology — Fri, 29 Oct 2010 20:59:32 +0100

Abstract (Source: The Journal of Pathology)

Residual Postoperative Tumour Volume Predicts Outcome after High-dose Radiotherapy for Chordoma and Chondrosarcoma of the Skull Base and Spine

Clinical Oncology — Wed, 27 Oct 2010 00:00:00 +0100

Conclusions: Our results show a high level of efficacy for fractionated photon radiotherapy after surgery, in keeping with other series. In addition, we found that although surgical debulking is essential, a small residual tumour volume may still be controlled with high-dose photon radiotherapy. This information may be relevant during neurosurgical planning, possibly allowing a reduction in risk of serious neurological deficits. This should encourage the further development of sophisticated photon radiotherapy, for patients unsuitable for proton therapy. (Source: Clinical Oncology)

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Implication for transcription factor T (brachyury) in the pathogenesis of sporadic chordoma: a genetic and functional‐based study

The Journal of Pathology — Wed, 20 Oct 2010 16:46:06 +0100

Abstract (Source: The Journal of Pathology)

Role of the transcription factor T (brachyury) in the pathogenesis of sporadic chordoma: a genetic and functional‐based study

The Journal of Pathology — Wed, 20 Oct 2010 00:00:00 +0100

In conclusion, chromosomal aberrations resulting in gain of the T locus are common in sporadic chordomas and expression of this gene is critical for proliferation of chordoma cells in vitro. Copyright © 2010 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd. (Source: The Journal of Pathology)

[Percutaneous intratumoral injection with pingyangmycin lipiodol emulsion for treatment of recurrent sacrococcygeal chordomas.]

Journal of Southern Medical University — Tue, 19 Oct 2010 23:00:00 +0100

CONCLUSION: Fluoroscopy-guided percutaneous intratumoral injection of PLE can be effective and safe and may serve as a alternative for treatment of recurrent sacrococcygeal chordomas. PMID: 20965848 [PubMed - as supplied by publisher] (Source: Journal of Southern Medical University)

Total resection of inferiorly located sacral chordoma with posterior only approach: case report and review of the literature.

Turkish Neurosurgery — Thu, 30 Sep 2010 23:00:00 +0100

Authors: Sabuncuoglu H, Ozdogan S, Dogan H, Ataoglu O, Tımurkaynak E Chordoma is a primary sacral neoplasm of ectodermal origin and makes up %1- 4 of all primary bone tumors. It is usually present on the midline cerebrospinal axis and the most common locations are the spheno-clival region and the sacrum. The treatment of primary sacral tumors represents a challenge because of a large tumor mass at presentation and a hemorrhage risk in surgery. Sacral tumors may present a difficult problem to the surgeon who desires to obtain a clear margin of excision. Using the retrorectal fat tissue as a cleavage line in the posterior approach guides the neurosurgeon to resect the tumor totally and reduce the hemorrhage in sacral chordomas. In this case report, we tried to discuss the advantages of ...

Fine needle aspiration cytology of primary or recurrent chordomas

Cytopathology — Wed, 29 Sep 2010 16:52:25 +0100

(Source: Cytopathology)

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Heidelberg Ion Therapy Center (HIT): Initial clinical experience in the first 80 patients.

Acta Oncologica — Sat, 25 Sep 2010 22:29:09 +0100

CONCLUSION: Particle therapy could be included successfully into the clinical routine at the Department of Radiation Oncology in Heidelberg. Numerous clinical trials will subsequently be initiated to precisely define the role of proton and carbon ion radiotherapy in radiation oncology. PMID: 20831505 [PubMed - in process] (Source: Acta Oncologica)

Proton Radiotherapy: The Biological Effect of Treating Alternating Subsets of Fields for Different Treatment Fractions

International Journal of Radiation Oncology * Biology * Physics — Mon, 02 Aug 2010 00:00:00 +0100

Purpose: Common practice in proton radiotherapy is to deliver a subset of all fields in the treatment plan on any given treatment day. We investigate using biological modeling if the resulting variation in daily dose to normal tissues has a relevant detrimental biological effect.Methods and Materials: For four patient groups, the cumulative normalized total dose (NTD) was determined for normal tissues (OARs) of each patient using the clinically delivered fractionation schedule (FSclin), and for hypothetical fractionation schedules delivering all fields every day (FSall) or only a single field each day (FSsingle). Cumulative three-dimensional NTD distributions were summarized using the generalized equivalent uniform dose (gEUD) model.Results: For the skull base/cervical spine chordoma group...

(ii) The investigation and radiological features of primary bone malignancy

Orthopaedics and Trauma — Sat, 31 Jul 2010 23:00:00 +0100

Abstract: Primary malignant bone tumours are rare. Imaging provides the foundation of surgical and oncological management as it offers a differential diagnosis for the nature of the lesion, defines its local extent and facilitates local-regional and distant staging. The role of radiographs, computed tomography (CT), magnetic resonance imaging (MRI) and bone scintigraphy is reviewed. In addition, some newer techniques such as whole-body MRI and FDG-PET imaging are discussed. Finally, we describe the epidemiology, subtypes and imaging features of the most common primary malignant bone tumours including osteosarcoma, chondrosarcoma, Ewing's sarcoma and chordoma. (Source: Orthopaedics and Trauma)

The Prognostic Value of Ki-67, p53, Epidermal Growth Factor Receptor, 1p36, 9p21, 10q23, and 17p13 in Skull Base Chordomas.

Archives of Pathology and Laboratory Medicine — Sat, 31 Jul 2010 13:54:43 +0100

Conclusions.-Chordomas with elevated Ki-67 proliferation index or deletion at 9p21 may be at risk for a more aggressive clinical course and shorter survival. These biomarkers may thus be used to improve therapeutic stratification. PMID: 20670138 [PubMed - in process] (Source: Archives of Pathology and Laboratory Medicine)

Revisiting chordoma with brachyury, a "new age" marker: analysis of a validation study on 51 cases.

Archives of Pathology and Laboratory Medicine — Sat, 31 Jul 2010 13:54:34 +0100

Conclusion.-Exclusive brachyury expression in more than 90% of chordomas indicates its value as a unique, specific marker with other sensitive markers like cytokeratin, epithelial membrane antigen, and/or S100 protein in substantiating a diagnosis of chordoma, including on small biopsies. PMID: 20670140 [PubMed - in process] (Source: Archives of Pathology and Laboratory Medicine)

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Proton Radiotherapy: The Biological Effect of Treating Alternating Subsets of Fields for Different Treatment Fractions.

Clinical Prostate Cancer — Fri, 30 Jul 2010 23:00:00 +0100

CONCLUSION: The effects of field set of the day treatment delivery depend on the tumor site and number of fields treated each day. Modeling these effects may be important for accurate risk assessment. PMID: 20675067 [PubMed - as supplied by publisher] (Source: Clinical Prostate Cancer)

Prognostic factors for long-term outcome of patients with surgical resection of skull base chordomas—106 cases review in one institution

Urosurgical Review — Wed, 28 Jul 2010 17:40:19 +0100

Abstract Skull base chordoma are still challenging. Between May 1993 and June 2005, 106 consecutive patients with skull base chordoma underwent surgical removal at Skull Base Division of Neurosurgery, Beijing Tiantan Hospital, China. Retrospective analysis included medical charts and images. The age of the patients ranged from 7 to 65 years old, with an average age of 35.6 years. Sixty patients were male; the other 46 were female (1.3:1). Follow up data were available in 79 cases ranging from 10 to 158 months (average 63.9 months) after operation. The prognostic factors for recurrence and survival were analyzed with Kaplan‐Meier, Cox regression and t‐test. Overall, 1, 3, 5 and 10 years survival rates were 87.2%, 79.4%, 67.6%and 59.5% respectively. One, 3, 5 and 10 year ...

Surgical Margins and Local Control in Resection of Sacral Chordomas.

Clinical Orthopaedics and Related Research — Thu, 15 Jul 2010 23:00:00 +0100

CONCLUSIONS: Surgical margins affect the risk of local recurrence. Previous intralesional surgery was associated with a higher rate of local recurrence. Intraoperative contamination did not affect the risk of local recurrence when wide margins were subsequently attained. LEVEL OF EVIDENCE: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence. PMID: 20635173 [PubMed - as supplied by publisher] (Source: Clinical Orthopaedics and Related Research)

Temporal Lobe Reactions After Radiotherapy With Carbon Ions: Incidence and Estimation of the Relative Biological Effectiveness by the Local Effect Model

International Journal of Radiation Oncology * Biology * Physics — Thu, 15 Jul 2010 23:00:00 +0100

Conclusions: Dmax,V − 1 cm3 is predictive for radiation-induced TLR. The tolerance doses obtained seem to be consistent with published data for highly conformal photon and proton irradiations. We could not detect any clinically relevant deviations between clinical findings and expectations based on predictions of the LEM. (Source: International Journal of Radiation Oncology * Biology * Physics)

Gain of chromosome 7 by chromogenic in situ hybridization (CISH) in chordomas is correlated to c-MET expression

Journal of Neuro-Oncology — Fri, 09 Jul 2010 06:18:21 +0100

Abstract Chordomas are low to intermediate grade malignancies that arise from remnants of embryonic notochord. They often recur after surgery and are highly resistant to conventional adjuvant therapies. Recently, the development of effective targeted molecular therapy has been investigated in chordomas that show receptors for tyrosine kinase (RTKs) activation. Expression of specific RTKs such as Epidermal Growth Factor Receptor (EGFR) and Mesenchymal-epithelial transition factor (c-MET) in chordomas may offer valuable therapeutic options. We investigated changes in copy number of chromosome 7 and correlated it with EGFR gene status and EGFR and c-MET protein expression in 22 chordoma samples. Chromosome 7 copy number was evaluated by chromogenic in situ hybridization ...

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[Protontherapy: basis, indications and new technologies.]

Bulletin du Cancer — Mon, 05 Jul 2010 23:00:00 +0100

Authors: Mazal A, Habrand JL, Delacroix S, Datchary J, Dendale R, Desjardins L, Ferrand R, Malka V, Fourquet A With over 70,000 patients treated worldwide, protontherapy has an evolution on their clinical applications and technological developments. The ballistic advantage of the Bragg peak gives the possibility of getting a high conformation of the dose distribution to the target volume. Protontherapy has accumulated a considerable experience in the management of selected rare malignancies such as uveal melanomas and base of the skull chordomas and chondrosarcomas. The growing interest for exploring new and more common conditions, such as prostate, lung, liver, ENT, breast carcinomas, as well as the implementation of large pediatric programs advocated by many experts has been challeng...

Sphenoid Masses in Children: Radiologic Differential Diagnosis with Pathologic Correlation [REVIEW ARTICLE]

American Journal of Neuroradiology — Thu, 01 Jul 2010 00:00:00 +0100

Congenital chordoma of the skin in a lamb (first report)

Comparative Clinical Pathology — Thu, 17 Jun 2010 11:26:45 +0100

Abstract Chordoma is a rare tumor in human beings and animals that originates from mesoderm-derived notochord. Although many cases of chordoma have been reported in human, dog, cat, rat, mink, and ferret, no report has been previously described in ruminants. This case report describes the clinical and histopathological findings of a congenital chordoma located in the occipital skin area of a 6-day-old, male, Iranian fat-tailed lamb. Histopathologic examination showed non-encapsulated tumor within the deep dermis. The neoplastic tissue consisted of physaliphorous cells with a fine fibrovascular stroma. The physaliphorous cells were immunoreactive with antibodies against vimentin, pancytokeratin, and S100 protein. Therefore, chordoma was diagnosed. To the best of the authors...

Expression of vascular endothelial growth factor and matrix metalloproteinase-9 in sacral chordoma

Journal of Neuro-Oncology — Wed, 16 Jun 2010 07:17:28 +0100

This study was designed to investigate the expression levels and contributions of vascular endothelial growth factor (VEGF) and matrix metalloproteinase-9 (MMP-9) in the angiogenesis and recurrence of sacral chordoma and their correlations. An immunohistochemical method was used to investigate the expression of VEGF, MMP-9, and microvascular density (MVD) in 36 patients with sacral chordoma. Their differences in expressions were statistically analyzed and their correlations with angiogenesis and recurrence were evaluated. The mean MVD of sacral chordomas was significantly higher than that of the adjacent normal tissues (P = 0.033). Immunoreactivity for VEGF and MMP-9 was significantly higher in sacral chordoma tissues than in adjacent normal tissues (P = 0.008, P&...

Chordoma of the sacrum: “En bloc” high partial sacrectomy

European Spine Journal — Tue, 01 Jun 2010 06:47:34 +0100

Content Type Journal ArticleCategory Open Operating Theatre (OOT)DOI 10.1007/s00586-010-1459-xAuthors Peter Paul VargaAron Lazary Journal European Spine JournalOnline ISSN 1432-0932Print ISSN 0940-6719 (Source: European Spine Journal)

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Chordoma of the sacrum: “en bloc” total sacrectomy and lumbopelvic reconstruction

European Spine Journal — Tue, 01 Jun 2010 06:47:18 +0100

Content Type Journal ArticleCategory Open Operating Theatre (OOT)DOI 10.1007/s00586-010-1460-4Authors Peter Paul VargaAron Lazary Journal European Spine JournalOnline ISSN 1432-0932Print ISSN 0940-6719 (Source: European Spine Journal)

Humerus metastasis from a sacrococcygeal chordoma: a case report

BioMed Central — Thu, 27 May 2010 23:00:00 +0100

Conclusion: Chordoma is a rare tumor and rarely metastasizes, but it should be considered in differential diagnosis of epithelioid bone tumors. The only currently effective treatment for this tumor is carbon ion therapy. There is currently no effective medical therapy available for advanced chordoma and the tumor is poorly sensitive to radiotherapy. (Source: BioMed Central)

Heavy ion radiotherapy during pregnancy

Fertility and Sterility — Wed, 19 May 2010 23:00:00 +0100

Objective: To provide a safe particle therapy treatment for a pregnant woman with skull-base cancer.Design: Case report.Setting: University clinic.Patient(s): A 27-year-old woman diagnosed for a skull-base chordoma and whose pregnancy was found during the course of radiotherapy with accelerated carbon ions.Intervention(s): Therapy was continued as scheduled, and fetal dose produced by photons and neutrons was measured at each radiotherapy fraction using passive and active monitors.Main Outcome Measure(s): Radiation dose to the uterus. Health of the mother and the newborn.Result(s): Total dose to the uterus was (Source: Fertility and Sterility)

Chordoma coexisting with Rathke's cleft cyst: Case report and literature review

Neuropathology — Tue, 18 May 2010 23:00:00 +0100

Both chordoma and Rathke's cleft cyst are relatively rare diseases in the central nervous system. In this paper we report the first case of a chordoma coexisting with a Rathke's cleft cyst. A 49-year-old man presented with a 19-month history of distending pain, movement dysfunction and diplopia of the left eye. The preoperative diagnosis was consistent with chordoma with cystic change. Final pathological diagnosis of chordoma coexisting with Rathke's cleft cyst was made according to histological and immunohistochemical studies and the clinical and radiological features are discussed. Considering the close relationship between the notochordal tissue and Rathke's pouch during early embryogenic development, a possible mechanism is also discussed with the literature review. (Source: Neuropatho...

Middle Turbinate Preservation in Endoscopic Transsphenoidal Surgery of the Anterior Skull Base

Skull Base — Tue, 11 May 2010 13:31:51 +0100

Skull BaseDOI: 10.1055/s-0030-1253582ABSTRACT Endoscopic endonasal skull base surgery is a growing field in which the nasal corridors are used to address skull base lesions. Whether the middle turbinates must be removed for adequate exposure is controversial and not well addressed in the literature. This is a prospective, observational study of 163 consecutive cases of purely endoscopic endonasal transsphenoidal surgeries performed at a single tertiary care institution. The primary study outcome measurement is the feasibility of middle turbinate preservation in endoscopic transsphenoidal skull base surgery. The pathologies included 99 pituitary tumors, 15 craniopharyngiomas, 11 meningiomas, 11 Rathke's cleft cysts, 7 encephaloceles, 5 cerebrospinal fluid leak repairs, 9 clival chordomas, a...

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Chordoid meningioma: a clinicopathologic study of 11 cases at a single institution

Journal of Neuro-Oncology — Sun, 09 May 2010 09:49:00 +0100

Abstract Chordoid meningioma is an uncommon variant of meningioma, which histologically bears a great resemblance to chordoma and often follows an aggressive clinical course. We examine clinicopathologic features of 11 cases of this rare tumor to further elucidate its behavior. Thirteen specimens of chordoid meningioma belonging to 11 patients were obtained at a single institution from 1995 to 2009. Correlations of histologic parameters, immunohistochemical study, and clinical features were assessed. This series included six men and five women with a mean age of 60.8 years at first surgery. Aside from one patient (case 5) who died of disease immediately after the first operation, the mean postoperative follow-up period for the other 10 patients was 41.4 months. T...

Unexpected sudden death due to intracranial chordoma: An autopsy case

Forensic Science International — Wed, 05 May 2010 23:00:00 +0100

We report an autopsy case of a sudden unexpected death due to clinically undiagnosed intracranial chordoma in the brainstem without haemorrhage. A 44-year-old man was found dead at home. The autopsy revealed two small gelatinous and semi-translucent greyish tumours on the ventral surface of the brainstem between the midbrain and pons. The tumours were not attached to the dura or bone. The brain was markedly swollen, with enlarged lateral and third ventricles, but the aqueduct was compressed and narrowed. Microscopically, the tumour invaded the cerebral peduncles, and was classified as a chordoma. The cause of death was diagnosed as acute obstructive hydrocephalus due to a ventral brainstem tumour. Even a minor intracranial tumour that is not primarily life-threatening may cause sudden deat...

Derivation and Characterization of an Extra-Axial Chordoma Cell Line (EACH-1) from a Scapular Tumor

JBJS [Am] — Mon, 03 May 2010 18:57:17 +0100

Conclusions: The diagnosis of the extra-axial chordoma is difficult but can be resolved by the detection of a strong brachyury expression. In addition, the derivation of a human extra-axial chordoma cell line could be a useful tool for the basic research of this rare neoplasm. Clinical Relevance: We describe the importance of brachyury staining in the diagnosis of this tumor. The extra-axial chordoma cell line (EACH-1) may also provide a new research tool to establish new ways of diagnosing and treating this rare malignancy. (Source: JBJS [Am])

Surgical excision of extensive sacrococcygeal chordomas assisted by occlusion of the abdominal aorta.

Journal of Neurosurgery.Spine — Fri, 30 Apr 2010 23:00:00 +0100

CONCLUSIONS: Occlusion of the abdominal aorta and electrophysiological monitoring are useful methods for assisting in resection of sacrococcygeal chordoma. They can reduce intraoperative hemorrhage and entail little chance of tumor cell contamination. They can also help surgeons to protect the organs in the pelvic cavity and neurological function. Use of these methods could give patients better quality of life. PMID: 20433296 [PubMed - in process] (Source: Journal of Neurosurgery.Spine)

Derivation and characterization of an extra-axial chordoma cell line (EACH-1) from a scapular tumor.

The Journal of Bone and Joint Surgery. American volume — Fri, 30 Apr 2010 23:00:00 +0100

CONCLUSIONS: The diagnosis of the extra-axial chordoma is difficult but can be resolved by the detection of a strong brachyury expression. In addition, the derivation of a human extra-axial chordoma cell line could be a useful tool for the basic research of this rare neoplasm. PMID: 20439670 [PubMed - in process] (Source: The Journal of Bone and Joint Surgery. American volume)

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Primary intradural chordoma: report on three cases and review of the literature.

Clinical Neuropathology — Fri, 30 Apr 2010 16:52:09 +0100

Conclusion: Considering our cases in context with so far published literature, we conclude that intradural chordomas are rare and in this location usually have a better prognosis compared to classical intraosseous chordomas. PMID: 20423692 [PubMed - in process] (Source: Clinical Neuropathology)

[Is proton beam therapy the future of radiotherapy? Part I: Clinical aspects.]

Cancer Radiotherapie — Sun, 25 Apr 2010 23:00:00 +0100

Authors: Bouyon-Monteau A, Habrand JL, Datchary J, Alapetite C, Bolle S, Dendale R, Feuvret L, Helfre S, Calugaru V, Cosset JM, Bey P Proton beam therapy uses positively charged particles, protons, whose physical properties improve dose-distribution (Bragg peak characterized by a sharp distal and lateral penumbra) compared with conventional photon-based radiation therapy (X-ray). These ballistic advantages apply to the treatment of deep-sited tumours located close to critical structures and requiring high-dose levels. [60-250 MeV] proton-beam therapy is now widely accepted as the "gold standard" in specific indications in adults - ocular melanoma, chordoma and chondrosarcoma of the base of skull - and is regarded as a highly promising treatment modality in the treatment of paediatric m...

Treatment with high marginal dose is mandatory to achieve long-term control of skull base chordomas and chondrosarcomas by means of stereotactic radiosurgery

Journal of Neuro-Oncology — Sat, 24 Apr 2010 08:06:57 +0100

This study was performed to evaluate the effect of stereotactic radiosurgery (SRS) for skull base chordomas and chondrosarcomas and to determine the optimal marginal dose for these tumors. Fourteen patients with histologically proven chordomas or chondrosarcomas underwent 16 sessions of SRS using gamma knife. The marginal doses ranged from 10 to 20 Gy (mean, 15 Gy). Lower marginal doses of 12 Gy on average (range, 10–12.5 Gy) were applied to four patients since they underwent prior fractionated radiotherapy, and partial treatment for which parts of tumors were excluded from planned target volume because of their proximity to critical structures was also applied to four patients. The whole tumors were covered with higher marginal doses of 18 Gy on average (r...

[Is CT-guided biopsy of aggressive sacral tumors always able to reach a diagnosis?]

Radiologia — Tue, 20 Apr 2010 23:00:00 +0100

CONCLUSION: Our results suggest that CT-guided biopsy is less useful in the characterization of primary tumors than in metastases. The diagnosis of primary bone lymphomas is especially complicated. Repeating the procedure after inconclusive results probably will not provide additional information, and it is recommendable to perform surgical biopsy in these cases. PMID: 20416912 [PubMed - as supplied by publisher] (Source: Radiologia)

Clinical Outcome of Sacral Chordoma With Carbon Ion Radiotherapy Compared With Surgery

International Journal of Radiation Oncology * Biology * Physics — Mon, 19 Apr 2010 00:00:00 +0100

Conclusion: Carbon ion RT results in a high local control rate and preservation of urinary-anorectal function compared with surgery. (Source: International Journal of Radiation Oncology * Biology * Physics)

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Image-guided, endoscopic, transcervical resection of cervical chordoma.

Journal of Neurosurgery.Spine — Wed, 31 Mar 2010 23:00:00 +0100

Authors: Hsu W, Kosztowski TA, Zaidi HA, Gokaslan ZL, Wolinsky JP Chordomas are rare tumors that arise from the sacrum, spine, and skull base. Surgical management of these tumors can be difficult, given their locally destructive behavior and predilection for growing near delicate and critical structures. En bloc resection with negative margins can be difficult to perform without damaging adjacent structures and causing significant clinical morbidity. For chordomas of the upper cervical spine, surgical options traditionally involve transoral or submandibular approaches. The authors report the use of the image-guided, endoscopic, transcervical approach to the upper cervical spine as an alternative to traditional techniques for addressing upper cervical spine tumors, particularly for tumo...

Bone versus soft-tissue sarcomas in the elderly.

Journal of Orthopaedic Surgery — Wed, 31 Mar 2010 23:00:00 +0100

CONCLUSION: Survival rates tend to be lower in older patients with sarcomas, especially when the sarcoma is of bone and high grade. PMID: 20427836 [PubMed - in process] (Source: Journal of Orthopaedic Surgery)

Proton Beam Radiation Therapy for Head and Neck Malignancies

Current Oncology Reports — Mon, 29 Mar 2010 17:15:24 +0100

Abstract Proton beam radiation therapy (PBRT) has unique physical properties (e.g., Bragg Peak) that limit the amount of normal tissue irradiated in the head and neck region while maximizing the radiation delivered to the tumor. Radiation therapy is commonly used in both the primary and adjuvant setting for many head and neck malignancies. Limiting the unnecessary radiation to normal tissues within the head and neck region can result in a profound improvement in quality of life during and after treatment. Although PBRT was initially developed in the 1950s, recent technological advances have permitted the development of hospital-based facilities for proton delivery. PBRT has been shown to improve outcomes for patients with sinonasal tumors, chordomas, chondrosarcomas, ocula...

Fine-needle aspiration of a lumbar chordoma

Diagnostic Cytopathology — Fri, 26 Mar 2010 00:00:00 +0100

No abstract. (Source: Diagnostic Cytopathology)

[A rare case of nasal obstruction: Clival chordoma.]

Presse Medicale — Fri, 26 Mar 2010 00:00:00 +0100

Authors: Saint-Blancard P, Weber-Donat G, Briche T, Rousselet T PMID: 20347574 [PubMed - as supplied by publisher] (Source: Presse Medicale)

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Lumbar vertebral chordoma arising from an intraosseous benign notochordal cell tumour: radiological findings and histopathological description with a good clinical outcome.

The British Journal of Radiology — Mon, 01 Mar 2010 00:00:00 +0100

We report a rare case of lumbar vertebral chordoma that was thought to arise from a benign intraosseous notochordal cell tumour and which showed significant osteosclerotic change. Radiologically, the lumbar vertebral mass lesion showed hyperintensity on T(2) weighted images, with scanty enhancement on post-contrast T(1) weighted MR images. High uptake corresponding to the mass was noted on fluorine-18-fluorodeoxyglucose positron emission tomography. Bone biopsy revealed proliferation of the physaliphorous cells between thickened bone trabeculae; no nuclear mitosis was observed. Although the mass was diagnosed clinically as spinal chordoma, histopathology contained both benign notochordal cell tumour and conventional chordoma. After heavy particle ((11)C)-charged radiation therapy was appli...

Novel Immunohistochemical Markers in the Diagnosis of Nonglial Tumors of Nervous System

Advances in Anatomic Pathology — Mon, 01 Mar 2010 00:00:00 +0100

Immunohistochemistry (IHC) has become an important adjunct tool in diagnostic neuro-oncology practice enabling immunophenotypic characterization of tumor cells. There have been several recent publications regarding new IHC markers that are useful for diagnosis of brain tumors. To introduce the latest advances in IHC in this field, we review the features of novel IHC marker antibodies applicable to selected nonglial tumors in the nervous system, based on recently published reports and our own experiences. We discuss (1) aquaporin-1 and [alpha]-inhibin for hemangioblastoma, (2) [beta]-catenin for craniopharyngioma, (3) brachyury for chordoma, and (4) INI-1 for hereditary schwannomas. All the markers presented here are used primarily for supporting or confirming the histologic diagnosis, with...

Endoscopic management of sphenoclival neoplasms: Anatomical correlates and patient outcomes

Otolaryngology - Head and Neck Surgery — Sat, 20 Feb 2010 14:20:24 +0100

Conclusion: The sphenoclival region poses a significant surgical challenge given its central location at the skull base and proximity to critical structures. This study demonstrates that transnasal endoscopic access to the sphenoclival region is technically feasible and allows successful surgical extirpation of tumors with a low complication rate and acceptable patient outcomes. (Source: Otolaryngology - Head and Neck Surgery)

Primary Chordoma in the Nasal Cavity and Nasopharynx: CT and MR Imaging Findings [HEAD AND NECK]

American Journal of Neuroradiology — Thu, 11 Feb 2010 16:01:13 +0100

CONCLUSIONS: Although no imaging features are pathognomonic, primary chordomas without skull base (clivus) bony changes in the nasal cavity and nasopharynx have some CT and MR imaging findings that are suggestive of diagnosis. The differential diagnosis of the soft tissue mass should be limited to these sites. (Source: American Journal of Neuroradiology)

Chordoma with increased prolactin levels (pseudoprolactinoma) mimicking pituitary adenoma: A case report with review of the literature

Journal of Cancer Research and Therapeutics — Thu, 11 Feb 2010 13:47:35 +0100

Kumar Pavan, Kumar Piyush, Singh Shalini, Kumari Niraj, Datta N RJournal of Cancer Research and Therapeutics 2009 5(4):309-311The article deals with a rare case of chordoma with increased prolactin levels. It could often result in a diagnostic dilemma and problems in differentiating it from a pituitary adenoma. (Source: Journal of Cancer Research and Therapeutics)

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Methylthioadenosine phosphorylase and activated insulin-like growth factor-1 receptor/insulin receptor: potential therapeutic targets in chordoma

The Journal of Pathology — Sat, 06 Feb 2010 00:00:00 +0100

Currently there is no effective chemotherapy for chordoma. Recent studies report co-expression of insulin-like growth factor-1 receptor (IGF1R) and its cognate ligand in chordoma, but it is unknown whether this receptor tyrosine kinase is activated in these tumours. Additionally, genetic studies have confirmed frequent deletions of chromosome 9p in chordomas, which encompasses the cyclin-dependent kinase inhibitor 2A (CDKN2A) locus. Another gene in this region, methylthioadenosine phosphorylase (MTAP), is an essential enzyme of the purine salvage pathway and has therapeutic relevance because MTAP-deficient cells are particularly sensitive to inhibitors of de novo purine synthesis. We investigated whether these pathways might be potential therapeutic targets for chordoma. Paraffin-embedded ...

Intraosseous benign notochordal cell tumor.

Archives of Pathology and Laboratory Medicine — Mon, 01 Feb 2010 00:00:00 +0100

Authors: Amer HZ, Hameed M Abstract Intraosseous benign notochordal cell tumor is a presumably benign, intravertebral lesion of notochord origin, which can be found incidentally on removal of the vertebra for unrelated lesions or during an autopsy. The notion of a larger benign notochordal cell tumor that is macroscopic, occasionally symptomatic, and seen radiologically was recently introduced, and described as giant vertebral notochordal rest, giant notochordal hamartoma of intraosseous origin, and benign chordoma . Because of the location, size, and similar histologic and immunohistochemical characteristics, larger benign notochordal cell tumors are easily misdiagnosed as the malignant notochord-type of tumor/chordoma, with critical implications. In this review, we attempt to shed li...

Vascularised fibula osteocutaneous flap for cervical spinal and posterior pharyngeal wall reconstruction

Indian Journal of Plastic Surgery — Fri, 29 Jan 2010 13:56:47 +0100

We report a case of vascularised fibula osteocutaneous flap used for composite cervical spinal and posterior pharyngeal wall reconstruction, in a patient with recurrent skull base chordoma, resected by an anterior approach via median labio-mandibular glossotomy approach. Bone stability and pharyngeal wall integrity were simultaneously restored (Source: Indian Journal of Plastic Surgery)

Chordoma: clinical characteristics, management and prognosis of a case series of 25 patients.

BMC Cancer — Thu, 28 Jan 2010 00:00:00 +0100

Conclusions: Despite progress of surgical techniques and the results obtained with targeted therapy, more effort is needed for better disease control. Specific experience of the multidisciplinar therapeutic team is, however, essential to succeed in improving patients' outcome. (Source: BMC Cancer)