Choi Myoung Eun, Lim Dong Jun, Chang Sung Eun, Lee Mi Woo, Choi Jee Ho, Lee Woo Jin
Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Ann Dermatol. 2020 Oct;32(5):426-429. doi: 10.5021/ad.2020.32.5.426. Epub 2020 Sep 29.
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.
假性肌源性血管内皮瘤（PMH）是一种罕见的血管肿瘤，最近被确认为一种独特的实体。它好发于年轻男性成年人，常发生于四肢远端。尽管已知其病程进展缓慢，但多灶性表现和局部复发很常见。PMH应与上皮样肉瘤、上皮样血管内皮瘤、隆突性皮肤纤维肉瘤和横纹肌肉瘤相鉴别。其特征性的免疫组化染色模式和复发性易位t(7:19)(q22:q13)是其诊断的基础。手术切除是主要的治疗方法，尽管对于无法手术的患者可考虑化疗。我们报告一例罕见病例，一名40岁的韩国男性患者通过切除活检被诊断为PMH，以促进在临床实践中对PMH的认识。
