Cutaneous Epithelioid Clear Cells Angiosarcoma in a Young Woman with Congenital Lymphedema

Flore Tabareau-Delalande,Anne-Valérie Decouvelaere,Gonzague De Pinieux,Elodie Miquelestorena-Standley,Anne De Muret

doi:10.1155/2013/931973

Flore Tabareau-Delalande, Anne-Valérie Decouvelaere + Show 3 more

Open Access

https://doi.org/10.1155/2013/931973

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Abstract

Angiosarcomas are rare aggressive neoplasms that can occur secondary to chronic lymphedema (Stewart-Treves syndrome). Although secondary angiosarcomas are commonly described after-mastectomy and/or after-radiotherapy, few cases have been reported in association with chronic lymphedema of congenital origin. We report the clinical, pathological, and cytogenetic findings in a case of cutaneous epithelioid clear cells angiosarcoma that occurred in a 21-year-old woman with hemibody congenital lymphedema. Surgical biopsies of the tumor mass revealed diffuse epithelioid proliferation of clear atypical cells, for which immunophenotyping highlighted the vascular differentiation. Despite en bloc resection of the tumor, the patient died of metastatic disease three months after diagnosis. This case illustrates the clinical and pathology characteristics of angiosarcoma that is a rare entity secondary to chronic lymphedema. It is the first reported case for which the c-MYC amplification status was assessed. The diagnostic value of this amplification should be further evaluated in this specific context.

Highlights

Angiosarcoma is an aggressive neoplasm that occurs in various soft tissues and visceral organs
This report provides clinical, pathology, and cytogenetics findings in an epithelioid cutaneous angiosarcoma occurring in a specific context of chronic lymphedema in a young woman with hemibody congenital lymphedema
We report a case of cutaneous congenital lymphedemaassociated secondary angiosarcoma, the diagnosis of which was rendered difficult by its predominant poorly differentiated epithelioid clear cell morphology

Summary

Background

Angiosarcoma is an aggressive neoplasm that occurs in various soft tissues and visceral organs. The most common sites (in decreasing order) are the skin, the breast, deep soft tissues, visceral organs, and bones. When the tumor occurs in the deep soft tissues, the preferential locations are the lower extremities, followed by the arms, the trunk, the head, and the neck [2]. The preferential locations for cutaneous angiosarcoma are the head and the neck, the limbs and the trunk [3]. Chronic lymphedema can be congenital, as in Milroy’s disease. This report provides clinical, pathology, and cytogenetics findings in an epithelioid cutaneous angiosarcoma occurring in a specific context of chronic lymphedema in a young woman with hemibody congenital lymphedema

Case Presentation

Pathology Findings

Discussion