Department of Surgical Pathology, Tata Memorial Hospital, MUMBAI, INDIA.
Turk Patoloji Derg. 2022;38(1):73-78. doi: 10.5146/tjpath.2021.01539.
This case report aims to present clinicopathological features of an extremely rare case of multifocal pseudomyogenic hemangioendothelioma (PMHE) in the scalp. A 21-year-old male developed multiple, focally ulcerated, nodules over the root of his nose and scalp. One of the skin lesions was sampled at another dermatology clinic, where this was diagnosed as a sarcoma. A review of biopsy sections showed well-circumscribed dermal lesions, comprising plump spindle and epithelioid cells, mimicking rhabdomyoblasts. Immunohistochemically, tumor cells were positive for AE1/AE3, CD31, FLI-1 and ERG. INI-1 was retained. A diagnosis of PMHE was offered. Subsequently, the patient underwent wide excision and has been asymptomatic for 8 months, post-surgery. PMHE is rarely reported in the head and neck region, where it can constitute a diagnostic pitfall. Awareness of this tumor and appropriate immunohistochemical stains are necessary for its timely diagnosis, in order to avoid radical treatments. A review of similar, previously documented cases is presented.
本病例报告旨在呈现头皮多灶性假肌源性血管内皮瘤（PMHE）这一罕见病例的临床病理特征。一名 21 岁男性在鼻根和头皮处出现多个局灶性溃疡性结节。其中一处皮损曾在另一家皮肤科诊所取样，当时被诊断为肉瘤。对活检切片进行复查后发现，界限清楚的真皮病变由丰满的梭形和上皮样细胞组成，类似于横纹肌母细胞。免疫组化染色显示，肿瘤细胞表达 AE1/AE3、CD31、FLI-1 和 ERG。INI-1 保持完整。因此，提出了 PMHE 的诊断。随后，患者接受了广泛切除，术后 8 个月无症状。PMHE 在头颈部罕见，可能构成诊断陷阱。为了避免激进治疗，及时诊断需要对此肿瘤和适当的免疫组化染色有一定认识。本文还对之前记录的类似病例进行了回顾。
