Metastatic alveolar soft part sarcoma masquerading as primary CNS tumor

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 disease-free 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 twenty-year-old 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 far. Keywords: Alveolar soft part sarcoma, brain metastasis, TFE3 nuclear positivity.