上肢假肌源性血管内皮瘤：1例报告并文献复习

[Pseudomyogenic hemangioendothelioma in the upper limb: A case report and literature review].

作者信息

Mayoral-Guisado Carlos, Toro-Zambrano Weimar, López-Macías María, Ruíz-Guerrero Antonio, Rubio-Fernández Alejandro, Díaz-Delgado Mario

机构信息

Servicio de Anatomía Patológica, Complejo Hospitalario Universitario de Badajoz, Badajoz, España.

出版信息

Rev Esp Patol. 2017 Jan-Mar;50(1):49-53. doi: 10.1016/j.patol.2015.12.007. Epub 2016 Mar 11.

DOI:10.1016/j.patol.2015.12.007

PMID:29179965

Abstract

Pseudomyogenic hemangioendothelioma, also called epithelioid sarcoma-like hemangioendothelioma, is a rare, vascular neoplasm usually with indolent behaviour. It was introduced in the latest World Health Organization (WHO) Classification of Tumours of Soft Tissue. We report a case of a 45 year-old patient presenting with a localized, palpable and slightly painful lesion in the left arm. Histologically it consisted of fascicles of spindle and epithelioid cells with ample eosinophilic cytoplasm, without nuclear pleomorphism or significant mitotic activity. Tumour cells showed diffuse expression for cytokeratin AE1/AE3, CD31 and FLI1, intact expression for INI1 and negativity for CD34. We describe the clinical, histological, molecular and immunohistochemical features of pseudomyogenic hemangioendothelioma and review the pertinent literature.

摘要

假肌源性血管内皮瘤，也称为上皮样肉瘤样血管内皮瘤，是一种罕见的血管肿瘤，通常行为惰性。它被纳入最新的世界卫生组织（WHO）软组织肿瘤分类中。我们报告一例45岁患者，其左臂出现一个局限性、可触及且稍有疼痛的病变。组织学上，它由梭形细胞和上皮样细胞束组成，具有丰富的嗜酸性细胞质，无核异型性或显著的有丝分裂活性。肿瘤细胞显示细胞角蛋白AE1/AE3、CD31和FLI1弥漫性表达，INI1表达完整，CD34阴性。我们描述了假肌源性血管内皮瘤的临床、组织学、分子和免疫组化特征，并复习相关文献。