骨上皮样血管肉瘤2例并文献复习

Epithelioid angiosarcoma of the bone: two case reports and literature review

采用光镜及免疫组织化学(immunohistochemistry,IHC)对2例骨上皮样血管肉瘤(epithelioid ang iosarcoma,E A S)进行回顾性分析并复习文献。肿瘤组织在镜下呈巢状、条索状排列,部分瘤细胞质内可见空泡、印戒样,其内有时可见红细胞,瘤细胞异型性较大,核仁明显,凋亡、坏死普遍,核分裂多见。免疫组织化学上皮样细胞CD31,CD34,CK7,波形蛋白(vimentin)弥漫阳性,抗细胞角蛋白单克隆抗体(AE1/AE3)部分阳性;CD45,Cg A,突触素(synaptophysin,Syn)、CD56,CK5/6,CK20,甲状腺转录因子1(thyroid transcription factor 1,TTF-1),尿路上皮特异蛋白III基因(Uroplakin III)、前列腺特异抗原(prostate specific antigen,PSA),p504s,CDX2均阴性。骨EAS是一种高度恶性肿瘤,易被误诊为恶性上皮样肿瘤。其鉴别诊断包括转移性腺癌、上皮样血管内皮瘤(epithelioid hemangioendothelioma,EHE)、上皮样血管瘤。最有效的治疗方法是手术切除,如有必要,可联合化疗或放疗。

The clinical characteristics and the microscopic and immunohistochemical characteristics of two cases with epithelioid angiosarcoma of bone were analyzed with follow-up period. Related literature was reviewed. Microscopically, the tumors cell were arranged in nests and cords; the neoplastic cells showed severe nuclear atypia and prominent eosinophilic cytoplasm with vacuoles, attempting to form primitive vasculature. Red blood cells are sometimes seen inside. Nucleolus is obvious, apoptosis and necrosis are common, can see some Mitotic. Immunohistochemically, tumor cells were positive to CD31, CD34, CK7 and vimentin. AE1/AE3 was weakly positive and CD45, Cg A, Syn, CD56, CK5/6, CK20, TTF-1, Uroplakin III, PSA, p504 s, CDX2 were negative. Epithelioid angiosarcoma represents a high-grade type of aggressive tumor. It can be misdiagnosed as malignant epithelioid tumors. The differential diagnosis of the epithelioid angiosarcoma includes metastatic adenocarcinoma, epithelioid hemangioendothelioma and epithelioid hemangioma. The most effective treatment is surgical resection, if necessary, combined with chemotherapy or radiotherapy.