甲状腺髓样癌16例临床病理分析

Medullary carcinoma of thyroid: a clinicopathologic study of 16 cases

目的 探讨甲状腺髓样癌 (MTC)的临床病理学特点。方法 对 16例经病理诊断为甲状腺髓样癌患者的临床资料、免疫组化、组织化学及光镜进行回顾性分析与研究。结果 患者大多以颈部肿块就诊 ,可伴腹泻症状。术后 5年、10年生存率分别为 85. 7%和 5 7.1%。散发型甲状腺髓样癌 15例 ,家族型甲状腺髓样癌 1例。癌组织中降钙素 (CT)阳性 16例、CgA阳性 15例、NSE阳性 10例、Syn阳性 7例、3例见甲状腺球蛋白 (Tg)阳性细胞 ,淀粉样物质染色均阳性。结论 MTC具有多分化肿瘤的特点 ,可以表达多种神经内分泌标志物 ,少数可出现类癌综合征或顽固性腹泻。其诊断依赖于组织病理学、免疫组化和组织化学 ,并须结合其临床资料。

Objective To investigate the clinical and pathological characteristics of medullary thyroid carcinoma (MTC). Methods The clinicopathologic data were collected in 16 cases of MTC confirmed by pathology. The tumors were retrospectively analyzed by light microscopy, histochemistry and immunohistochemistry. Results The initial syndrome in the most patients was the cervical mass and diarrhea. The survival rates of 5 and 10 years were 85.7% and 57.1%, respectively. Sporadic type of MTC was found in 15 cases, and familial type in one case. Calcitonin (CT) immunoreactive cells were present in all the tumors. Chromogranin A (CgA) was present in 15 cases. 10 tumors contained neuron-specific enolase (NSE) immunoreactive cells. 7 cases were positive for synaptophysin (Syn). Thyroglobulin (TG) was present in three tumors. There was amyloid in the stroma of all cases. Conclusions MTC has characteristics of the tumor with multiple differentiation, which can express numerous hormones, and even manifest carcinoid syndrome or refractory diarrhea. Its diagnosis depends on its histopathology, immunohistochemistry and histochemistry. Its clinical manifestation should also be taken into account. Radical dissection is the principal treatment of MTC.