A rare case of primary clear cell sarcoma of the leg in an elderly
Journal Title: MedPulse -International Medical Journal - Year 2014, Vol 1, Issue 3
Abstract
Clear cell sarcoma (CCS) is an aggressive, rare soft-tissue tumor representing approximately 1 per cent of all soft tissue sarcomas. Also called malignant melanoma of soft parts because it shares a melanocytic phenotype that include presence of melanin, ultra structural evidence of melanosomes, and immunohisto chemical staining for S-100 protein and melanoma associated antigen HMB-45. Diagnostic difficulties arise with metastatic malignant melanoma to soft tissue. DNA ploidy analysis reveals that CCS has a diploid or lesser degree of an euploidy when compared with malignant melanoma. This paper reports on a clear cell sarcoma occurring at a very old age in upper part of leg. The tumor consisted of round to spindle cells with clear cytoplasm and multinucleated giant cells. Both S100 and HMB45 were positive. DNA analysis by Flow Cytometry showed a diploid pattern of growth. Patient underwent wide excision of the tumor. The case supports the contention that distinctive histology, IHC and DNA ploidy analysis were helpful to delineate CCS. Wide excision with negative margins remains the mainstay of treatment.
Authors and Affiliations
Monoj Kumar Deka, Anuradha Talukdar, Ritesh Tapkire, Rajeev Kumar, Akalesh K Verma, Ravi Kannan
Keywords
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