骨原发性上皮样血管肉瘤6例临床病理学分析

Primary epithelioid angiosarcoma of bone:a clinicopathological study of six cases

目的探讨骨原发性上皮样血管肉瘤的临床病理特征、诊断与鉴别诊断。方法分析6例骨原发性上皮样血管肉瘤临床资料,观察其组织学形态及免疫表型,并复习相关文献。结果本组男性5例,女性1例,中位年龄46.5岁。其中发生于股骨2例、肱骨2例、胫骨1例、髋骨1例。临床症状均为病变部位疼痛,组织学上,肿瘤组织由片状或条索状排列的上皮样细胞构成,部分区域可见大小不等、形态不规则分支状血管性腔隙,内衬异型瘤细胞,部分瘤细胞内见含红细胞的空泡。免疫组化均不同程度表达CD31、vimentin,部分表达ERG、Fli-1、CKpan。4例获得随访资料,其中2例死亡,2例无复发。结论骨原发性上皮样血管肉瘤罕见,必须与转移癌等鉴别,诊断需结合临床及影像学资料配合免疫组化可以明确诊断。

Objective To explore the clinicopathological features, diagnosis and differential diagnosis of primary epithelioid angiosarcoma of bone （PEAB）. Methods By analysis of the clinical data in 6 cases of PEAB, the histological morphology and immunohistochemical characteristics of PEAB were studied, and their relevant literatures were reviewed. Results Of 6 PEAB patients, there were 5 males and 1 female and median age was 46. 5 years. There were 2 cases in the femur, 2 cases in the humerus, 1 case in the tibia, 1 case in the hip bone. The symptom of these cases were localized pain. Histologically, the tumor consisted of solid sheets of large epithelioid cells. Vasoformative structures ranged from well-formed vessel to slit-like anastomosing vascular channels were seen in local region. Vacuoles containing red blood cells were also noted in some tumor cells. Immunohistochemically, the tumor cells were stained positive for CD31, vimentin, partly postive for ERG, Fli-1 and CKpan. Of the 6 cases, follow-up data were available for 4 patients, 2 of them were died after surgical resection; the remaining two were survival. Conclusions Primary epithelioid angiosarcoma of bone is very rare, and it should be different from metastatic carcinoma. An accurate diagnosis is based on clinical manifetations, imaging data and immunohistochemical staining.