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
Soft tissue sarcoma is associated with various rare cancers, which includes clear cell sarcoma, angiosarcoma, alveolar soft-part sarcoma, malignant mesenchymoma, adult fibrosarcoma, clear cell sarcoma, rhabdomyosarcoma, and kaposi sarcoma.
The differential diagnosis of MNTI includes other small round blue cell tumors of childhood, especially neuroblastoma, Ewing sarcoma, alveolar rhabdomyosarcoma, and lymphoma, in addition to melanogenic tumors, including malignant melanoma and clear cell sarcoma of soft tissue.
Because of the unusual histologic presentation, which included the presence of clear cells, multinucleated giant cells, and with no epidermal in situ component, the differential diagnosis included clear cell sarcoma and Spitz nevus.
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 .
The most useful tests in reevaluating diagnosis were FISH (for assessing MDM2 amplification status to determine whether differentiated lipomatous tumours or pleomorphic tumours were ALT/WDL or DDL, resp.) and for assessment of EWSR1 gene rearrangement (for diagnosis of a variety of tumours, such as clear cell sarcoma).