CLEAR CELL SARCOMA IN A CHILD- A RARE ASSOCIATION

Dr. Shristi Butta*, Dr. Neepamanjari Barman, Dr. Moumita Maiti, Dr. Ranu Sarkar

ABSTRACT

Clear cell sarcoma, also known as malignant melanoma of the soft parts, accounts for 1% of all soft tissue neoplasms. It has a predilection for the distal extremities. It occurs in the age group of 2040yrs and is rare in the pediatric population. The tumour is thought to arise from neural crest cells and expresses various markers of melanoma. The tumour also shows a characteristic chromosomal translocation giving rise to the transcript EWS-ATF1 gene which helps to distinguish it from malignant melanoma. Here we present a case of clear cell sarcoma in a 10 year old child, who presented with a swelling over the left knee joint of 6 months duration. Imaging revealed a soft tissue mass without any bone involvement. Histopathology from the mass showed cells with round to oval nuclei and prominent nucleoli arranged in nests and fascicles, surrounded by thin fibrous septa & interspersed with multinucleated giant cells. Immunohistochemistry revealed positivity for HMB-45, thus confirming the diagnosis of Clear cell sarcoma of the tendon sheath.

Keywords: clear cell sarcoma, child, rare, melanoma, translocation, immunohistochemistry.