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|LETTER TO EDITOR
|Year : 2011 | Volume
| Issue : 6 | Page : 929-931
Extraskeletal intracranial mesenchymal chondro-sarcoma: Report of a rare case
VG Ramesh1, Shantha Ravisankar1, R Vimal Chander2, P Saminathan3
1 Institute of Neurology, Madras Medical College, Tambaram, Chennai, India
2 Institute of Pathology, Madras Medical College, Tambaram, Chennai, India
3 Bharat Specialities Lab, Tambaram, Chennai, India
|Date of Submission||31-Aug-2011|
|Date of Decision||20-Sep-2011|
|Date of Acceptance||10-Oct-2011|
|Date of Web Publication||2-Jan-2012|
Institute of Neurology, Madras Medical College, Tambaram, Chennai
|How to cite this article:|
Ramesh V G, Ravisankar S, Chander R V, Saminathan P. Extraskeletal intracranial mesenchymal chondro-sarcoma: Report of a rare case. Neurol India 2011;59:929-31
|How to cite this URL:|
Ramesh V G, Ravisankar S, Chander R V, Saminathan P. Extraskeletal intracranial mesenchymal chondro-sarcoma: Report of a rare case. Neurol India [serial online] 2011 [cited 2013 May 24];59:929-31. Available from: http://www.neurologyindia.com/text.asp?2011/59/6/929/91392
Extraskeletal intracranial mesenchymal chondrosarcoma is a rare, aggressive malignant neoplasm arising from the meninges or brain parenchyma. Liechtenstein and Bernstein in 1959 first described mesenchymal chondrosarcoma as a variant of chondrosarcoma.  We report a rare case of extraskeletal mesenchymal chondrosarcoma presenting as temporal lobe mass in a young girl.
A 15-year-old girl presented with sudden onset of severe headache, nausea and vomiting. Computer tomography (CT) cranial scan showed a left frontoparietal hyperdense space-occupying lesion with hemorrhage and midline shift [Figure 1]. She had craniotomy and removal of the lesion in fragments. Gross examination showed multiple grayish brown soft tissue fragments of varying sizes (0.2 cm to 2 cm) measuring about 5 cc in aggregate. Hematoxylin and eosin-stained sections revealed a biphasic tumor composed of a population of undifferentiated small round cells and spindle to oval cells with hyperchromatic nuclei. Few foci show hemangiopericytoma-like pattern with staghorn blood vessels [Figure 2] and [Figure 3]. In the midst of the tumor cells, there were islands of benign-appearing cartilage. Immunostaining showed S100 positivity [Figure 4] in the cartilaginous islands while epithelial membrane antigen was negative in the tumor cells, thus ruling out meningioma. Vimentin was positive [Figure 5] . The final impression was extraskeletal mesenchymal chondrosarcoma. After six months, she presented again with severe headache and CT scan revealed a left temporoparietal mass lesion with perilesional edema and midline shift. She had second craniotomy and resection of the mass. Histopathology revealed features of mesenchymal chondrosarcoma, suggestive of recurrence.
|Figure 2: Mesenchymal chondrosarcoma showing undifferentiated cells and benign-appearing cartilage ×200|
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Mesenchymal chondrosarcoma is a rare high-grade malignant neoplasm of unknown etiology characterized by islands of hyaline cartilage and undifferentiated small round blue cells. This lesion accounts for 1-3% of all chondrosarcomas and 30% of the lesions occur in extraskeletal sites.  Head, neck and lower extremities are the common extraskeletal sites, of which the central nervous system is the most common site, especially the meninges. It presents in the second and third decades with no gender predilection. , Signs and symptoms mostly depend on the location of the lesion and local infiltration.
Macroscopically, the size of the tumor ranges from 2.5-37 cm  and presents as a well-defined, multilobulated, soft to firm, gray-white to brown in color. Cut surface shows gray-white solid areas with pale blue cartilaginous areas with or without calcification. Occasional areas show cystic degeneration, hemorrhage or necrosis. Light microscopy clearly depicts a dimorphic feature with well-defined islands of benign-looking cartilage and undifferentiated small round blue cells.  The transition between the two may be gradual or abrupt. Areas of hemangiopericytomatous pattern with staghorn-shaped vascular spaces with rare mitoses and necrosis are seen. Cartilaginous areas show S100 positivity but EMA negativity. Undifferentiated component shows positivity for Vimentin, neuron-specific enolase, Leu-7, Sox9, and CD99. In the absence of cartilaginous tissue, immunochemical markers will be less conclusive in differentiating from other small round-cell neoplasms. Typical microscopic features of mesenchymal chondrosarcoma help in easy diagnosis, nonetheless absence of chondroid features causes difficulties in diagnosing this condition. Sometimes, the presence of spindle-shaped undifferentiated cells with occasional whorling, calcification and absence of hemangiopericytomatous pattern mimics metaplastic meningioma, cartilaginous type.  However, careful observation of the pattern and cytomorphology of the tumor will be of help rather than immunohistochemical markers.
Long-term prognosis is poor, however. Surgical resection and radiation therapy to the tumor bed are recommended. Careful follow-up for recurrence is necessary. Chemotherapy and if possible surgical resection are the treatment of choice in recurrent cases.
| » References|| |
|1.||Lichten Stein L, Bernstein D. Unusual benign and malignant chondroid tumours of bone. A surgery of some mesenchymal cartilage tumours and malignant chondroblastic tumours, including a few multicentric ones, as well as many a typical benign chondroblastomas and chondromyxoid fibromas. Cancer 1959;12:1142-57. |
|2.||Tyagis, Maheshwari V, Tyagi N. Mesenchymal chondrosarcoma a case report. J Postgrad Med 1992;38:34-5. |
|3.||Scheuthaner BW, Rubinstein LJ. Meningeal mesenchymal chondrosarcoma: Report of 8 cases with review of the literature. Cancer 1978;42:2744-52. |
|4.||Rushing EJ, Mena H, Smirniotopoulos JG. Mesenchymal Chondrosarcoma of the cauda equina. Clin Neuropathol 1995;14:150-3. |
|5.||Reyaz N, Ashraf M. Intracranial extraskeletal Mesenchymal chondrosarcoma. J Ayub Med Coll Abbottabad 2006;18:86-7. |
|6.||Aigner T, Oliveira AM, Nasiemento AG. Extra skeletal myxoid chondrosarcoma do not show a chondrocytic pheno type. Mod Pathol 2004;17:214-21. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]