A sarcoma of young female adults, which affects the legs and acral regions. Clear cell sarcomas arise in tendons as circumscribed, but unencapsulated melanin-rich neuroectodermal tumours Prognosis 45–60% death, late recurrence
Clear cell sarcoma (CCS) is a rare distinctive soft tissue sarcoma, known to originate from neural crest cells, that has been classified as a variant of melanoma since it was first reported in 1965 .
Three-year-old Therese Faye Macababbad was diagnosed at Tawam Hospital, UAE's leading cancer hospital and a Johns Hopkins affiliate, with a clear cell sarcoma of the kidney on February 2010, when she was only 18 months old.
This method is sensitive yet not specific for ES/PNET because there are other entities that demonstrate EWS gene rearrangement, including desmoplastic small round cell tumor, clear cell sarcoma, extraskeletal myxoid chondrosarcoma, and myxoid liposarcoma.