家族性甲状腺乳头状癌的生物学特征及治疗

目的 分析家族性甲状腺乳头状癌的临床生物学行为,并探讨其治疗方法.方法 回顾性分析2010年1月至2011年8月天津医科大学附属肿瘤医院收治的家族性甲状腺乳头状癌共15个家系36例患者的临床资料,并将随机抽取的同期散发性甲状腺乳头状癌95例患者作为对照组.结果 15例(41.7％)家族性甲状腺乳头状癌患者年龄≥45岁;12例(33.3％)双侧腺叶发病,20例(55.6％)多灶病变;27例(75.0％)伴颈部淋巴结转移;17例(47.2％)伴甲状腺良性结节.95例对照组中60例患者(63.2％)年龄≥45岁;12例(12.6％)双侧腺叶发病,21例(22.1％)多灶病变;51例(53.7％)伴颈部淋巴结转移;26例(27.4％)伴甲状腺良性结节.家族性甲状腺乳头状癌患者行甲状腺全切除22例,患侧腺叶及峡部切除14例;3例行单侧或双侧侧颈清扫及中央区颈清扫术,7例行单侧或双侧后侧颈清扫及中央区颈清扫术,6例行一侧后侧颈清扫及中央区颈清扫术+对侧中央区颈清扫术,20例行单侧或双侧中央区颈清扫术.结论 家族性甲状腺乳头状癌较散发性甲状腺乳头状癌具有发病年龄小,多灶发病及双侧发病比例高,颈部淋巴结转移率高的生物学特征.术前应详细询问家族史并评估病情,建议选择较为积极的治疗方式.

Biological characteristics and management of familial papillary thyroid carcinoma

Objective To analyze the clinical biological characteristics and investigate the managements of familial papillary thyroid carcinoma (FPTC).Methods Clinical data of 36 patients with PTC from 15 families were retrospectively analyzed compared with 95 control cases taken randomly from the patients with sporadic PTC diagnosed and treated in Tianjin Cancer Hospital between January 2010 and August 2011.Results Of the 36 patients with FPTC,15 (41.7％) were ≥45 years old,12 (33.3％ ) had bilateral carcinoma,20 ( 55.6％ ) were multifocality,27 ( 75.0％ ) had neck lymph node metastases,17(47.2％ )coexisted thyroid benign tumors.Of the 95 patients with SPTC,60( 63.2％ ) were ≥45 years old,12( 12.6％ ) had bilateral carcinomas,21 ( 22.1％ ) were multifocality,51 ( 53.7％ ) had neck lymph node metastases,and 26 ( 27.4％ ) coexisted thyroid benign tumors.Of the 36 patients with FPTC,22 (61.1％) underwent total thyroidectomy and 14 (38.9％) with unilateral thyroidectomy plus isthmusectomy,3(8.3％ ) received unilateral or bilateral lateral neck dissection and central compartment neck dissection( CND),7 ( 19.4％ ) received unilateral or bilateral posterolateral neck dissection and CND,6 (16.6％) received posterolateral neck dissection and bilateral CND,and 20 (55.6％) received unilateral or bilateral CND.Conclusions Age at disease presentation of FPTC was younger than that of SPTC.FPTC has higher rates of multifocality and bilateral carcinoma coexisting with thyroid benign tumor than those of SPTC.It necessary to take family history in detail and to evaluate diseases before operation.