Primary Clear Cell Sarcoma of the Dermis Mimicking Malignant Melanoma.

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Citation: Balkan Medical Journal. 35(2):203-207, 2018 Mar 15PMID: 29072181Institution: MedStar Washington Hospital CenterDepartment: PathologyForm of publication: Journal ArticleMedline article type(s): Journal ArticleSubject headings: *In Situ Hybridization, Fluorescence | *Melanoma/di [Diagnosis] | *Sarcoma, Clear Cell/di [Diagnosis] | Adult | Female | Humans | Melanoma/pa [Pathology] | Sarcoma, Clear Cell/pa [Pathology]Year: 2018ISSN:
  • 2146-3123
Name of journal: Balkan medical journalAbstract: 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.All authors: Brenholz P, Obiorah IE, Ozdemirli MFiscal year: FY2018Digital Object Identifier: Date added to catalog: 2018-04-20
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Journal Article MedStar Authors Catalog Article 29072181 Available 29072181

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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