Recurrent clear cell sarcoma and its responsiveness to chemoradiation

Clear cell sarcomas are rare tumours accounting for less than 1% of all soft tissue tumours. They are aggressive lesions with extremely poor prognosis. The clinical diagnosis of this tumour is challenging, requiring histopathologic examination for definite characterization. The treatment protocol for this tumour is not well-established, with most studies showing no clear cut role of chemoradiation. This is a case of clear cell sarcoma of soft tissue in a 57-year-old male who presented with a solid-cystic mass on the thigh and was diagnosed as hematoma clinicoradiologically. Post-operative residual tumour was treated successfully with radio/chemotherapy, in a manner similar to melanoma. This article, thus, also highlights the role of chemoradiation in the treatment of clear cell sarcoma, suggesting that it can be better treated like melanoma rather than like a soft tissue sarcoma.