多发性内分泌腺瘤病1型合并垂体腺瘤的临床特点

Clinical features in patients with multiple endocrine neoplasia type 1 complicated with pituitary adenoma

目的探讨多发性内分泌腺瘤病1型（MEN1）患者中垂体腺瘤的发生情况及其临床特征。方法回顾性分析北京协和医院内分泌科1985年1月至2014年12月收治的92例MEN1患者中，合并垂体腺瘤患者的临床特点。结果92例中，65例（70．7％）MEN1患者被诊断合并垂体腺瘤，其中功能性垂体腺瘤39例（60．0％），无功能垂体腺瘤23例（35．4％），功能不详者3例（4．6％）。MEN1患者中，女性垂体腺瘤受累的比率为75．9％（41／54），男性为63．0％（24／38），差异无统计学意义（P=0．275）。功能性垂体腺瘤的平均起病年龄为（34．3±11．3）岁，其中最常见类型为催乳素腺瘤（48．7％，19／39），其次为混合型垂体腺瘤（25．6％，10／39）、促肾上腺皮质激素腺瘤（15．4％，6／39）及生长激素腺瘤（10．3％，4／39）。无功能垂体腺瘤中，微腺瘤的比率为60．9％（14／23），高于大腺瘤的34．8％（8／23）。结论MEN1患者常合并垂体腺瘤，故疑诊MEN1患者应常规进行垂体动态增强MRI检查；而垂体腺瘤患者要警惕合并MEN1的可能，需注意筛查。

Objective To investigate the occurrence of pituitary＂ adenoma and its clinical features in patients with multiple endocrine neoplasia type 1 （MEN 1 ）. Methods Clinical features of patients with 92 MEN 1 complicated with pituitary adenoma treated at the Department of Endocrinology, Peking Union Medical College Hospital from January 1985 to December 2014 were analyzed retrospectively. Results In 92 patients, 65 （70.7%） with MEN 1 were diagnosed as having pituitary adenoma, including 39 （60.0%） with functional pituitary adenoma, 23 （35.4%） with non-functional pituitary adenoma, and 3 （4.6%） with unknown function of pituitary adenoma. In patients with MEN 1, the ratio of fenmle involvement in pituitary adenomas was 75.9% （41/54） and that of male was 63. 0% （24/38）. There was no significant difference （P =0.275）. The mean onset age of the functional pituitary adenomas was 34. 3 ±11.3 years, and the most common type was prolactinomas （48.7%, 19/39）, followed by the mixed pituitary adenomas （25.6%, 10/39）, adrenoeorticotropic hormone adenomas （15. 4%, 6/39）, and somatotrophinomas （10. 3%, 4/39）. In the non-functional pituitary adenomas, the ratio of microadenomas was 60.9% （ 14/23 ）. It was higher than 34. 8% （ 8,/23 ） of the macroadenomas. Conclusions The MEN 1 patients often have pituitary adenomas, so patients with suspected MEN 1 should routinely conduct pituitary dynamic enhanced MRI examinations, and patients with pituitary adenoma should be alert to the possible combination of MEN 1 and should pay attention to screening.