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A Case of Pseudomyogenic Hemangioendothelioma of the Lower Extremity

Annals of Dermatology 2020년 32권 5호 p.426 ~ 429
최명은, 임동준, 장성은, 이미우, 최지호, 이우진,
소속 상세정보
최명은 ( Choi Myoung-Eun ) - University of Ulsan College of Medicine Asan Medical Center Department of Dermatology
임동준 ( Lim Dong-Jun ) - University of Ulsan College of Medicine Asan Medical Center Department of Dermatology
장성은 ( Chang Sung-Eun ) - University of Ulsan College of Medicine Asan Medical Center Department of Dermatology
이미우 ( Lee Mi-Woo ) - University of Ulsan College of Medicine Asan Medical Center Department of Dermatology
최지호 ( Choi Jee-Ho ) - University of Ulsan College of Medicine Asan Medical Center Department of Dermatology
이우진 ( Lee Woo-Jin ) - University of Ulsan College of Medicine Asan Medical Center Department of Dermatology

Abstract


Pseudomyogenic hemangioendothelioma (PMH) is a rare vascular tumor and was recently recognized as a distinct entity. It has a predilection for young male adults and it frequently occurs in distal extremities. Although it is known to follow an indolent course, multi-focal presentation and local recurrence are common. PMH should be differentiated from epithelioid sarcoma, epithelioid hemangioendothelioma, dermatofibrosarcoma protuberans, and rhabdomyosarcoma. Its characteristic immunohistochemical staining pattern and recurrent translocation t(7:19)(q22:q13) are the basis for its diagnosis. Surgical excision is the mainstay treatment, although chemotherapy can be considered in non-operable patients. We present a rare case of a 40-year-old Korean male patient diagnosed with PMH through an excisional biopsy to facilitate the recognition PMH in the clinical practice.

키워드

Epithelioid cells; Hemangioendothelioima; Leg; Soft tissue neoplasms

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