14_GovindMangal 324 Govind et al Primary intracranial extraosseus myxoid chondrosarcoma of dominant frontal lobe Primary intracranial extraosseus myxoid chondrosarcoma of dominant frontal lobe Mangal Govind1, Mittal Radheyshyam2, Sharma Achal3, Gandhi Ashok4 SMS medical college and hospitals Jaipur, India 1Mch resident in Neurosurgery; 2Professor and head of department, department of Neurosurgery; 3Professor and Faculty, department of Neurosurgery; 4Associate Professor and Faculty, department of Neurosurgery Introduction Primary intracranial extraosseus myxoid chondrosarcoma are an extremely rare neoplasm. Till date there are only nine cases of this entity has been reported. The authors present a case of primary intracranial extraosseus myxoid chondrosarcoma of the dominant frontal lobe. Case report A 40 years old male was admitted to our department with complaints of headache on and off and recurrent seizures for last five years. Patient was on antiepileptic drugs as advised by his treating physician and he responded well earlier but for last one year seizures were not in control hence he was investigated. On imaging, NCCT brain shows an ill-defined heterogenous mass in left frontal region with focal areas of calcification. MRI brain shows a well circumscribed intra-axial heterogeneously hypointense mass on T1W and hyperintense on T2W and FLAIR MRI sequence. There was minimal perilesional edema. No restriction of diffusion seen on DWI and diffusion coefficient was increased on ADC sequence. Faint patchy enhancement seen on contrast images. Left frontotemporoparietal craniotomy with complete excision of mass done. Tumor was well circumscribed greyish white in colour, firm to hard in consistency, relatively avascular, loosely adherent to surrounding brain parenchyma. A plane of cleavage was present at the lesion brain interface. On histopathology, sections shows circumscribed lobulated growth pattern with areas having hyaline cartilage. The lobules are variable in size. The capsule is moderately thickened and is showing mild lymphocytic infiltrate. The tumor has low to moderate cellularity and nuclear pleomorphism. Predominantly the lacunae are mononucleated however binucleated and multinucleated lacunae are also seen. Focal areas of calcification and fibromyxoid changes are also seen. Overall morphology is in favour of chondrosarcoma/chondroma grade -1. On immunohistochemistry tumor cells are strongly positive for vimentin. 50-75 percent cells shows Bcl-2 expression. CD99, P-53, E- cadherine, EMA, CK8/18 and CK PAN (AE1- AE3) are negative in the tumor cells. Postoperative period was uneventful and patient was discharged on fifth postoperative day without any neurologic deficit. Antiepileptics were initially prescribed for one month and then tapered gradually over fifteen days. On follow up of six months patient had no complaints and seizures were under control on phenytoin 300mg per day. Romanian Neurosurgery (2015) XXIX 3: 324 - 328 325 Figure 1 - NCCT brain shows an ill defined heterogenous mass in left frontal region with focal areas of calcification Figure 2 - MRI brain shows a well circumscribed intra-axial heterogeneously hyperintense on T2W imaging Figure 3 - Tumor was well circumscribed greyish white in colour, firm to hard in consistency, relatively avascular Figure 4 - Postoperative NCCT brain suggestive of left frontotemporoparietal craniotomy with complete excision of mass 326 Govind et al Primary intracranial extraosseus myxoid chondrosarcoma of dominant frontal lobe Figure 5 - showing cells are Bcl-2 positive Figure 6 - showing cells are S-100 positive (high power) Figure 7 - There is blue myxoid matrix located in lacunae. Tumor cells are lobulated and double nucleated cells are presentwith foci of calcification Discussion Since Enziner and Shiraki first described myxoid chondrosarcoma in 1972 as a deep soft tissue tumor of the extremities only nine cases of primary intracranial extraosseus myxoid chondrosarcoma have been reported as per authors’ knowledge. (2, 4, 5, 9, 10, 13, 15, 16, 17). Intracranial myxoid chondrosarcoma is extremely rare and is thought to arise from the choroid plexus, dura or in rare instances the pineal region. (1, 3, 6, 9, 14, 17). Histologically only three subtypes of intracranial chondrosarcomas have been described: classic chondrosarcoma, mesenchymal chondrosarcoma and myxoid chondrosarcoma. (5, 8, 11). Most of the Primary intracranial extraosseus chondrosarcoma show a dural involvement. However those within the brain parenchyma without any attachment to the cranium or the meninges are very rare. The case of author is of myxoid chondrosarcoma having no dural involvement. The classic cranial and intracranial chondrosarcoma usually arise at the skull base and most frequently affect adults. (8, 15). The classic subtype has a better prognosis than the mesenchymal subtype. (15). Mesenchymal type usually occurs in the frontoparietal region and is highly vascular (15) it is the most aggressive subtype with a tendency for recurrence and metastasis. (8, 15). Calcification was more common in cranial myxoid chondrosarcoma and it lacks hyaline. The optimal treatment for intracranial myxoid chondrosarcoma is radical excision, and total removal of the tumor is critical. (5, 8, 12, 14, 15). Adjuvant therapies, including radiotherapy, brachytherapy, and proton beam treatment, have been found to improve patient outcomes for this rare cancer. (5, 11, 15). However there have been no reports on the advantages of adjuvant chemotherapy for patients with intracranial extraskeletal myxoid chondrosarcomas till date. In addition, intracranial myxoid chondrosarcomas are not associated with a good prognosis after treatment. Romanian Neurosurgery (2015) XXIX 3: 324 - 328 327 Correspondence Dr. Govind Mangal Mch neurosurgery resident SMS medical college and hospitals Jaipur, India +91-9414728062 dr.govindmangal@gmail.com References 1.Bourgouin PM, Tampieri D, Robitaille Y, Robert F, Bergeron D, del Carpio R, et al.: J Comput Assist Tomogr 16: 268-273, 1992.10 2.Chaskis C, Michotte A, Goossens A, Stadnik T, Koerts G, D’Haens J: Primary intracerebral myxoid chondrosarcoma. Case illustration. J Neurosurg 97: 228, 2002.2 3.Cummings TJ, Bridge JA, Fukushima T: Extraskeletal myxoid chondrosarcoma of the jugular foramen. Clin Neuropathol 23: 232-237, 2004 . 11 4.Enzinger FM, Shiraki M: Extraskeletal myxoid chondrosarcoma. An analysis of 34 cases. 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