原发性甲状腺血管肉瘤临床病理观察

Clinicopathologic study of primary angiosarcoma of thyroid

目的探讨原发性甲状腺血管肉瘤的临床病理学特征。方法对3例原发性甲状腺血管肉瘤进行临床大体分析、组织形态学及免疫组织化学染色观察，结合文献对其临床病理特点及鉴别诊断进行探讨。结果患者男性2例，女性1例；年龄52～78岁。镜下肿瘤组织弥漫浸润甲状腺，肿瘤内可见不规则的相互吻合的血管腔，局灶血管腔内可见乳头状突起，部分肿瘤细胞实性片状排列。肿瘤细胞上皮样、梭形或多角形，胞质丰富、嗜酸性，细胞核圆形、空泡状，核仁明显。坏死与核分裂象显著。免疫组织化学染色显示肿瘤细胞不同程度阳性表达CD31、ERG、Fli1、CD34、第八因子相关抗原、广谱细胞角蛋白。结论原发性甲状腺血管肉瘤是罕见的高度恶性的肿瘤，易误诊为甲状腺未分化癌；熟悉其临床病理学特征并应用免疫组织化学血管内皮标志物检查可避免误诊。

Objective To investigate the clinicopathologic characteristics of primary angiosarcoma in thyroid. Methods Three cases of primary angiosarcoma in thyoid were analyzed by gross examination, light microscopy and immunohistochemieal staining, as well as related literatures were reviewed. Results The patients included 2 males and 1 female. The age of patients ranged from 52 to 78 years. The tumors showed an infiltrating histological pattern. Irregular, anastomosing gaping vascular channels were usually evident, and intravascular papillary fronds were occasionally present. In other areas, tumors featured a solid pattern of growth with cells arranged in sheets and nests with no intervening stroma. The neoplastic cells were epithelioid, spindled or polygonal-shaped with abundant eosinophilic cytoplasm and round, large, vesicular nuclei containing prominent nucleoli. The tumors showed brisk mitotic activity and necrosis. Immunohistochemical study revealed that the tumor cells were positive for CD31, ERG, Flil, CD34, FVIHRAg and CKpan to different extents. Conclusions Primary epithelioid angiosarcoma of the thyroid is a rare, high-grade malignancy that may be misdiagnosed as other malignancies, especially anaplastic thyroid carcinoma. Recognizing its clinieopathologie characters and combined application of specific vascular endothelial immunohistochemical markers is important to avoid confusion with other lesions.