Department of Pathology, Guangdong Provincial People's Hospital/Guangdong Academy of Medical Sciences, 106, Zhongshan Road II, Guangzhou, 510000, China.
Diagn Pathol. 2019 Jul 17;14(1):79. doi: 10.1186/s13000-019-0857-6.
Pseudomyogenic hemangioendothelioma (PHE)/epithelioid sarcoma-like hemangioendothelioma (ES-H) is a rare vascular tumor of intermediate malignancy that commonly occurs in soft tissue of distal extremities of young adults. PHE typically has a multifocal presentation and can involve several tissue planes, including the dermis, subcutis, muscle and bone.
We present here a unique case of PHE/ESH that arose in the breast as well as a review of the published literature. The initial biopsy was interpreted as a metaplastic carcinoma. However, complete resection largely revealed plump epithelioid cells, and a more spindled cell component was also noted. The cells displayed abundant eosinophilic cytoplasm and central vesicular nuclei arranged in loose fascicles, with a mild, mixed acute and chronic inflammatory infiltrate. Overall, linear membranous staining of CD31 and lack of CD34 expression were highly suggestive of PHE. At the same time, FOSB immunoreactivity was observed, which supported PHE/ESH instead of metaplastic carcinoma. The patient has not shown recurrence in the half year follow up after total mastectomy.
To our knowledge, this is the first report of breast involvement in this neoplasm. Recognition of its histopathological features and immunohistochemical reactivity will prevent misdiagnosis of breast lesions.
假肌源性血管内皮瘤（PHE）/上皮样肉瘤样血管内皮瘤（ES-H）是一种罕见的中度恶性血管肿瘤，常见于年轻成人四肢远端的软组织中。PHE 通常呈多灶性表现，并可累及多个组织平面，包括真皮、皮下组织、肌肉和骨骼。
我们在此介绍一例发生在乳房的 PHE/ES-H 独特病例，并对已发表的文献进行了回顾。最初的活检被解释为化生性癌。然而，完全切除后主要显示出饱满的上皮样细胞，也注意到了更梭形的细胞成分。这些细胞显示出丰富的嗜酸性细胞质和中央泡状核，呈松散的束状排列，伴有轻度混合性急性和慢性炎症浸润。总体而言，CD31 的线性膜染色和缺乏 CD34 表达强烈提示为 PHE。同时，观察到 FOSB 免疫反应性，支持 PHE/ES-H 而不是化生性癌。患者在全乳切除术后半年的随访中没有出现复发。
据我们所知，这是首例该肿瘤累及乳房的报告。认识其组织病理学特征和免疫组织化学反应将有助于防止对乳腺病变的误诊。
