家族性甲状腺非髓样癌临床分析

Clinical features of familial non-medullary thyroid cancer patients

目的分析家族性甲状腺非髓样癌的临床特征。方法收集2003年9月至2012年9月初治的家族性甲状腺非髓样癌60例作为研究组，随机抽取60例散发型甲状腺非髓样癌患者作为对照组，分析两组患者的临床资料。结果研究组和对照组患者均为甲状腺乳头状癌。研究组患者的发病年龄为21～66岁，中位年龄42．0岁；女40例，男20例；TI期16例，乃期37例，T4期7例。对照组患者的发病年龄为24～78岁，中位年龄45．5岁；女49例，男11例。T1期25例，他期2例，13期30例，T4期3例。研究组和对照组中累及甲状腺双叶者分别占43．3％（26/60）和18．3％（11/60），差异有统计学意义（P=0．003）。研究组和对照组中多灶发病者分别占53．3％（32/60）和35．O％（21/60），被膜或被膜外侵犯者分别占73．3％（44/60）和55．0％（33/60），合并良性病变者分别占81．7％（49/60）和50．0％（30/60），差异均有统计学意义（均P〈0．05）。研究组和对照组患者的淋巴结转移率分别为60．0％（36/60）和38．3％（23/60），差异有统计学意义（P=0．018）。研究组患者中亲子型32例，同胞型28例；亲子型中第一代发病中位年龄为58岁，第二代发病中位年龄为32岁，差异有统计学意义（P〈0．001）。结论家族性甲状腺非髓样癌尤其是亲子型，具有较高的侵袭性，易累及双叶，多灶发病，常合并甲状腺良性疾病。建议对原发灶行甲状腺全切除术，必要时术后行^132I治疗。对于家族性甲状腺非髓样癌家系中家属，建议〉20岁者行颈部超声筛查，以便早期发现和早期治疗。

Objective To analyze the clinical features of familial non-medullary thyroid cancers, and to discuss their management. Methods Sixty thyroid cancer patients with familial non-medullary thyroid cancer （FNMTC）history during Sep. 2003 to Sep. 2012 were collected as study group, and another 60 sporadic thyroid cancer patients were randomly chosen as control. We compared the differences of their clinical features. Results All the patients were diagnosed as thyroid papillary carcinoma. The study group included 40 female and 20 male patients. There were 16 cases in stage T1, 37 in stage T3, and 7 in stage T4. The patients were 21-66-year old （ median age 42-years）. The control group included 49 female and 11 male patients. The patients were 24- to 78-year old, and the median age was 45.5 years. 43.3% of the patients in the study group and 18.3% of the patients in the control group had bilateral carcinomas （P = 0.003 ）. There were multifocal lesions in 53.3% of the patients in the study group and 35.0% of the control group, local invasion in 73.3% of the patients in the study group and 55.0% of the control group, and coexisting benign thyroid diseases in 81.7% of the patients of the study group and 50.0% of the control group （P 〈0.05 for all）. There were cervical lymph node metastases in 60.0% of the patients in the study group and 38.3% in the control group （P =0. 018）. In the study group, 32 cases were of parent-offspring type and 28 cases of sibling type. Among the parent-offspring type patients, the median onset age of the first generation offsprings was 58 years, and that of the second generation offsprings was 32 years （P 〈 0.001 ）. Conclusions Familial nonmedullary thyroid cancer, especially in parent-offspring type patients, is more aggressive than sporadic nonmedullary thyroid cancer, and often involves bilateral lobes, has multifocality, and combines with benign thyroid diseases. We recommend a total thyroidectomy for treatment, and to screen all the family members 〉 20 years old, with uhrasonography for early diagnosis and treatment.