Metastatic alveolar soft part sarcoma masquerading as primary CNS tumor
Journal Title: Indian Journal of Neurosciences - Year 2018, Vol 4, Issue 3
Abstract
Alveolar soft part sarcoma ASPS is a very rare malignant highly aggressive and profusely vascular soft tissue sarcoma of controversial histological origin accounting for less than 1 of the soft tissue sarcoma cases worldwide Though it has an indolent clinical course however because of its propensity to recur and frequently metastasize it usually has a fatal outcome The knowledge of its microscopic features and TFE3 staining facilitate earlier diagnosis Surgical resection helps in increasing the diseasefree period but the role of adjuvant chemotherapy and radiotherapy is disputed After resection it should be frequently monitored because of its malignant potential and dismal prognosis We present a case report of twentyyearold male patient whose entire clinical presentation was that of a primary brain malignancy with no symptoms anywhere else in the body but was diagnosed to be a case of alveolar soft part sarcoma of para lumbar region with brain and lung metastasis To the best of our knowledge it is an extremely uncommon presentation with only fourteen cases have been reported so farKeywords Alveolar soft part sarcoma brain metastasis TFE3 nuclear positivity
Authors and Affiliations
Deepak K. Goel, Priti P. Trivedi, Shailee P. Mehta, Paresh G. Mody, Dipak D. Patel
Keywords
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