ISSN : 2146-3123
E-ISSN : 2146-3131

Primary Clear Cell Sarcoma of the Dermis Mimicking Malignant Melanoma
Ifeyinwa E. Obiorah1, Pauline Brenholz2, Metin Özdemirli1
1Department of Pathology, Medstar Georgetown University Hospital, Washington, USA
2Department of Medical Genetics, Integrated Oncology, New York, USA
DOI : 10.4274/balkanmedj.2017.0796

Background: Clear cell sarcoma is a rare malignant soft tissue neoplasm that typically involves tendons and aponeurosis. Clear cell sarcoma occurring in the dermis is an extremely rare occurrence. It is difficult to differentiate between this neoplasm and dermal malignant melanoma because they both have similar morphologic and immunohistochemical features. Although rare, clear cell sarcoma of the skin typically occur in the extremities. To our knowledge, there are no reported cases of primary clear cell sarcoma of the skin occurring in the neck. In this paper, we report an unusual case of clear cell sarcoma arising in the skin of the right neck.
Case Report: A 43-year-old female presented with a right neck lesion. Histologic sections of the lesion, showed a nodular proliferation of spindle cells with pale cytoplasm with epithelioid features involving the entire dermis with no epidermal component. The tumor cells were positive for melanocytic markers, including S100 and Human Melanoma Black 45 (HMB-45), which led to an initial diagnosis of malignant melanoma. Fluorescence in situ hybridization (FISH) showed a rearrangement of the EWSR1 gene on chromosome 22q12, which led to a diagnosis of primary clear cell sarcoma in the skin.
Conclusion: Since the treatment for clear cell sarcoma and conventional melanoma are very different, FISH for EWSR1 should be performed in any dermal lesions with melanocytic features that do not have in situ component.

Keywords : Clear cell, melanoma, dermis, sarcoma
Viewed : 213
Downloaded : 0