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目的 探索家族性甲状腺乳头状癌的临床特征、诊断标准和处理原则。 方法 在随机的 145例甲状腺乳头状癌患者中进行前瞻性的调查共发现家族性甲状腺乳头状癌有 7个家族 17例患者。其中 14例患者为本院手术 ,3例外院手术。 17例患者病理均经本院病理科证实。占同期的甲状腺乳头状癌的 9 6 5 % (14/145 )。 结果 本组年龄 30～ 74岁 ,平均年龄 45岁 ;原发灶大小 0 8～2 8cm ,平均 1 7cm ;单侧 9例 ,双侧 8例 (4 7 5 % ) ;3个家族同时有 3例患病 (4 2 8% ) ;有 4个家族的成员患有甲状腺良性肿瘤和非肿瘤性甲状腺疾病。 17例患者中伴有结节性甲状腺肿的 10例。全甲状腺切除 8例 ,腺叶加峡部切除 9例 ;行甲状腺癌联合根治术的 6例 ;14例有不同区域的淋巴结转移 ,占 82 3% (14/17)。 结论 家族性甲状腺乳头状癌占甲状腺乳头状癌 10 %左右 ;双侧病变近 5 0 % ,淋巴结转移率高 ;处理基本同于散发性甲状腺乳头状癌。但是 ,患病家族成员应长期随访
Objective To explore the clinical features, diagnostic criteria and treatment principles of familial thyroid papillary carcinoma. Methods A prospective study of 145 randomly selected patients with papillary thyroid carcinoma found 17 patients with familial papillary thyroid carcinoma in seven families. Of these, 14 were hospital-based and 3 were hospital-based. The pathology of 17 patients was confirmed by our hospital pathology department. Accounting for 96.5% (14/145) of thyroid papillary carcinomas in the same period. Results The age ranged from 30 to 74 years, with an average age of 45 years. The size of the primary tumor ranged from 0 to 28 cm (mean, 17 cm). There were 9 unilateral and 8 bilateral (47.5% Cases of illness (42.8%); members of four families with thyroid benign and non-neoplastic thyroid disease. Thirteen patients had nodular goiter in 10 cases. There were 8 cases of total thyroidectomy, 9 cases of adenomyosis and isthmus resection, 6 cases of thyroid cancer combined with radical mastectomy, and 14 cases of lymph node metastasis in different regions (82.3%, 14/17). Conclusion Familial papillary thyroid carcinoma accounts for about 10% of papillary thyroid carcinoma; bilateral lesions of nearly 50%, lymph node metastasis rate is high; treatment is basically the same with sporadic thyroid papillary carcinoma. However, the affected family members should be followed up for a long time