VandenBussche Christopher J, Wakely Paul E, Siddiqui Momin T, Maleki Zahra, Ali Syed Z
Department of Pathology, The Johns Hopkins Hospital, Baltimore, Md., USA.
Acta Cytol. 2014;58(4):356-66. doi: 10.1159/000366151. Epub 2014 Sep 5.
Epithelioid hemangioendothelioma (EHE) and epithelioid angiosarcoma (EAS) are rare vascular neoplasms that share many morphological characteristics on histology but demonstrate different clinical behavior. Given the many reported clinical and morphological features shared between EAS and EHE, we examined all cases of EAS and EHE diagnosed primarily on fine needle aspiration (FNA) at our three institutions that were confirmed by a tissue diagnosis.
A total of 29 cases from 25 patients were identified: 15 EHE from 11 patients and 14 EAS from 14 patients.
Many cytomorphological features existed on a spectrum that overlapped considerably between EAS and EHE cases. Common features between the two entities include epithelioid morphology and eccentrically placed nucleus. Intracytoplasmic lumens (ICL), a morphological feature that may suggest vascular origin, can be found in both entities but are not always present.
Given the general absence of vascular cytomorphological features, such as ICL, the proper classification of these tumors depends on the successful use of immunoperoxidase markers such as factor VIII-related antigen, ERG, or CD31 as well as a high index of suspicion. The distinction between EAS and EHE on FNA alone is treacherous at best.
上皮样血管内皮瘤(EHE)和上皮样血管肉瘤(EAS)是罕见的血管肿瘤,在组织学上具有许多形态学特征,但临床表现不同。鉴于EAS和EHE之间有许多已报道的临床和形态学特征,我们检查了在我们三家机构主要通过细针穿刺活检(FNA)诊断并经组织学确诊的所有EAS和EHE病例。
共鉴定出25例患者的29例病例:11例患者的15例EHE和14例患者的14例EAS。
EAS和EHE病例在细胞形态学特征方面存在很大程度的重叠。两者共有的特征包括上皮样形态和偏位核。胞质内管腔(ICL)是一种可能提示血管起源的形态学特征,在两种肿瘤中均可发现,但并非总是存在。
鉴于通常缺乏血管细胞形态学特征,如ICL,这些肿瘤的正确分类取决于免疫过氧化物酶标记物(如因子VIII相关抗原、ERG或CD31)的成功应用以及高度的怀疑指数。仅靠FNA区分EAS和EHE极具难度。