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
Highlights
Clear Cell Sarcoma (CCS) of soft tissue, previously referred to as melanoma of soft parts is a rare malignant tumor arising from soft tissues around tendons and aponeurosis of distal extremities
Clear appearance of cytoplasm is due to accumulation of intracellular glycogen which can be demonstrated with periodic acid-Schiff (PAS) and PAS with diastase (PAS-D) stains
Apart from malignant melanoma another differential diagnosis that should be kept in mind while evaluating a tumor located in proximity to tendons and aponeurosis includes paraganglioma-like dermal melanocytic tumour, clear cell myelomonocytic tumour, epithelioid malignant peripheral nerve sheath tumour and monophasic synovial sarcoma
Summary
Clear Cell Sarcoma (CCS) of soft tissue, previously referred to as melanoma of soft parts is a rare malignant tumor arising from soft tissues around tendons and aponeurosis of distal extremities. We are describing here a case of 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. FNAC smears revealed metastatic deposits of tumour composed of neoplastic oval to spindle shaped cells arranged in small clusters and scattered singly, having prominent red inclusion-like nucleoli. The presence of these oval to spindle shaped cells with large nucleoli raised the suspicion of melanoma. (Fig 1) in view of soft tissue lesion around the knee, the possibility of metastasis from clear cell sarcoma was suggested. The patient was started on cisplatin and doxorubicin-based chemotherapy; he expired within two days of initiation of therapy
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