Clear cell sarcoma of right knee with bone marrow metastasis: A case report and review of literature.

  • Divya Shelly Armed Forces Medical College, Pune
  • Shashank Mishra AFMC,Pune
  • Divya Gupta AFMC,Pune
  • Reena Bharadwaj AFMC, Pune
Keywords: clear cell sarcoma, soft tissue, amelanotic melanoma, bone marrow metastasis

Abstract

Clear cell sarcoma (CCS) of the soft tissue is an extremely rare malignant tumor which was first described by Dr Franz M Enzinger in 1965. Soft tissue sarcomas comprise only 1% of malignant tumors and clear cell sarcoma is the rarest among these. It is closely associated with tendons, aponeurosis and fascial structures of extremities. Histomorphologically and immunohistochemically it resembles amelanotic melanoma and was earlier called as malignant melanoma of soft parts. But it is a unique soft tissue tumour with uninvolved overlying skin and characteristic chromosomal translocation. The characteristic histopathological features along with immunohistochemical positivity for S-100p, Melan-A and HMB-45 help in differentiation of this tumour from other lesions like fibrosarcoma and MPNST. The common site of metastasis includes lymph nodes, lungs, skin, liver, heart and brain.  We present a rare case of an 18-year-old boy with clear cell sarcoma of right knee, which had lymph node metastasis at presentation and found to have bone marrow metastasis during the course of the disease. DOI: 10.21276/apalm.2017.1107

Author Biographies

Divya Shelly, Armed Forces Medical College, Pune
Assistant Professor
Shashank Mishra, AFMC,Pune
Department of Pathology, Resident
Divya Gupta, AFMC,Pune
Department of Pathology, Resident
Reena Bharadwaj, AFMC, Pune
Department of pathology, Head of department.

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Published
2017-02-17
Section
Case Report

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