Ifeyinwa E OBİORAH, Pauline BRENHOLZ, Metin ÖZDEMİRLİ

Primary Clear Cell Sarcoma of the Dermis Mimicking Malignant Melanoma

Background: Clear cell sarcoma is a rare malignant soft tissue neoplasm that typically involves tendons and aponeurosis. Clear cell sarcoma in the dermis is an extremely rare occurrence, and it is difficult to differentiate between this neoplasm and dermal malignant melanoma because they have similar morphologic and immunohistochemical features. Although rare, clear cell sarcoma of the skin typically occurs in the extremities. To our knowledge, there are no reported cases of primary clear cell sarcoma of the skin occurring in the neck. Here, we report an unusual case of clear cell sarcoma arising in the skin of the 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 tumour cells were positive for melanocytic markers, including S100 and Human Melanoma Black 45, which led to an initial diagnosis of malignant melanoma. Fluorescence in situ hybridization showed a rearrangement of the EWSR1 gene on chromosome 22q12, which led to a diagnosis of primary clear cell sarcoma in the skin. Conclusion: Because the treatments for clear cell sarcoma and conventional melanoma are different, fluorescence in situ hybridization for EWSR1 should be performed in any dermal lesions with melanocytic features that do not have an in situ component.

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1. Enzinger FM. Clear-Cell Sarcoma of Tendons and Aponeuroses. An Analysis of 21 Cases. Cancer 1965;18:1163-74.
2. Chung EB, Enzinger FM. Malignant melanoma of soft parts. A reassessment of clear cell sarcoma. Am J Surg Pathol 1983;7:405-13.
3. Falconieri G, Bacchi CE, Luzar B. Cutaneous clear cell sarcoma: report of three cases of a potentially underestimated mimicker of spindle cell melanoma. Am J Dermatopathol 2012;34:619-25.
4. Hantschke M, Mentzel T, Rütten A, Palmedo G, Calonje E, Lazar AJ, et al. Cutaneous clear cell sarcoma: a clinicopathologic, immunohistochemical, and molecular analysis of 12 cases emphasizing its distinction from dermal melanoma. Am J Surg Pathol 2010;34:216-22.
5. Gambichler T, Pantelaki I, Othlinghaus N, Moritz RK, Stricker I, Skrygan M. Deep intronic point mutations of the KIT gene in a female patient with cutaneous clear cell sarcoma and her family. Cancer Genet 2012;205:182-5.
6. Kiuru M, Hameed M, Busam KJ. Compound clear cell sarcoma misdiagnosed as a Spitz nevus. J Cutan Pathol 2013;40:950-4.
7. Langezaal SM, Graadt van Roggen JF, Cleton-Jansen AM, Baelde JJ, Hogendoorn PC. Malignant melanoma is genetically distinct from clear cell sarcoma of tendons and aponeurosis (malignant melanoma of soft parts). Br J Cancer 2001;84:535-8.
8. Mavrogenis A, Bianchi G, Stavropoulos N, Papagelopoulos P, Ruggieri P. Clinicopathological features, diagnosis and treatment of clear cell sarcoma/melanoma of soft parts. Hippokratia 2013;17:298-302.
9. Dim DC, Cooley LD, Miranda RN. Clear cell sarcoma of tendons and aponeuroses: a review. Arch Pathol Lab Med 2007;131:152-6.
10. Hisaoka M, Ishida T, Kuo TT, Matsuyama A, Imamura T, Nishida K, et al. Clear cell sarcoma of soft tissue: a clinicopathologic, immunohistochemical, and molecular analysis of 33 cases. Am J Surg Pathol 2008;32:452-60.
11. Hocar O, Le Cesne A, Berissi S, Terrier P, Bonvalot S, Vanel D, et al. Clear Cell Sarcoma (Malignant Melanoma) of Soft Parts: a Clinicopathologic Study of 52 Cases. Dermatol Res Pract 2012;2012:984096.
12. Antonescu CR, Nafa K, Segal NH, Dal Cin P, Ladanyi M. EWS-CREB1: a recurrent variant fusion in clear cell sarcoma association with gastrointestinal location and absence of melanocytic differentiation. Clin Cancer Res 2006;12:5356-62.
13. Feasel PC, Cheah AL, Fritchie K, Winn B, Piliang M, Billings SD. Primary clear cell sarcoma of the head and neck: a case series with review of the literature. J Cutan Pathol 2016;43:838-46.
14. Sidiropoulos M, Busam K, Guitart J, Laskin WB, Wagner AM, Gerami P. Superficial paramucosal clear cell sarcoma of the soft parts resembling melanoma in a 13-yearold boy. J Cutan Pathol 2013;40:265-8.
15. Schwede K, Wetzig T, Simon JC, Fischer L, Wickenhauser C, Scharer L, et al. Cutaneous clear cell sarcoma in a 12-year-old boy. J Dtsch Dermatol Ges 2013;11:757- 9.
16. Park BM, Jin SA, Choi YD, Shin SH, Jung ST, Lee JB, et al. Two cases of clear cell sarcoma with different clinical and genetic features: cutaneous type with BRAF mutation and subcutaneous type with KIT mutation. Br J Dermatol 2013;169:1346- 52.