Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.
China Pituitary Disease Registry Center, Chinese Pituitary Adenoma Specialist Council, Beijing, 100730, China.
Pituitary. 2019 Apr;22(2):113-123. doi: 10.1007/s11102-019-00939-x.
To explore the clinical characteristics of pituitary adenomas in patients with MEN1 and to summarize treatment strategies for MEN1 in a Chinese population.
We retrospectively analyzed 54 MEN1 patients with pituitary adenomas diagnosed at Peking Union Medical College Hospital from March 2003 to January 2017. Clinical data, laboratory testing results, treatments of involved glands and treatment responses were collected and analyzed.
The mean age at pituitary adenoma diagnosis was 53.9 ± 17.8. The patients initially consulted the Endocrinology, General Surgery and Neurosurgery departments, in descending frequency. The nonfunctioning adenoma, prolactinoma, GH-secreting adenoma, cosecreting adenoma, and ACTH-secreting adenoma subtypes accounted for 48.1%, 27.8%, 9.3%, 9.3% and 5.6% of the cases, respectively. The remission rate for prolactinomas was 46.2% (6/13) treated with bromocriptine. And the remission rates were 87.5% (7/8) and 100% (3/3) for GH-secreting adenomas and ACTH-secreting adenomas respectively achieved by transsphenoidal surgery. Nineteen (35.2%) patients with asymptomatic nonfunctioning pituitary adenomas showed no progression after a 35-month follow-up with close observation. Regarding treatment priority, patients with thymic carcinoid tumors received first-line surgery, 54% of the patients with enteropancreatic tumors had these tumors treated first, and 26% of all patients had their pituitary adenomas treated first. In acromegalic patients, pituitary lesions tended to be treated first (75%, p = 0.002). PHPT and adrenocortical adenomas can be managed with elective surgery.
The treatment of MEN1 requires cooperation between multidisciplinary teams. Individualized treatment according to the severity of glandular involvement is needed. GH-secreting and ACTH-secreting pituitary adenomas require active treatment, while nonfunctioning pituitary adenomas can be observed closely.
探讨多发性内分泌腺瘤 1 型（MEN1）患者垂体腺瘤的临床特征，并总结中国人群中 MEN1 的治疗策略。
我们回顾性分析了 2003 年 3 月至 2017 年 1 月在北京协和医院诊断的 54 例 MEN1 伴垂体腺瘤患者的临床资料、实验室检查结果、累及腺体的治疗方法和治疗反应。
垂体腺瘤诊断时的平均年龄为 53.9±17.8 岁。患者最初依次咨询内分泌科、普通外科和神经外科。无功能腺瘤、催乳素瘤、生长激素（GH）分泌腺瘤、共分泌腺瘤和促肾上腺皮质激素（ACTH）分泌腺瘤分别占 48.1%、27.8%、9.3%、9.3%和 5.6%。溴隐亭治疗催乳素瘤的缓解率为 46.2%（6/13）。经蝶窦手术治疗 GH 分泌腺瘤和 ACTH 分泌腺瘤的缓解率分别为 87.5%（7/8）和 100%（3/3）。19 例（35.2%）无症状无功能垂体腺瘤患者经 35 个月密切观察后无进展。在治疗优先顺序方面，胸腺类癌患者行一线手术，54%的胰肠肿瘤患者先进行肿瘤治疗，26%的患者先进行垂体腺瘤治疗。在肢端肥大症患者中，倾向于先治疗垂体病变（75%，p=0.002）。甲状旁腺功能亢进症和肾上腺皮质腺瘤可采用择期手术治疗。
MEN1 的治疗需要多学科团队合作。需要根据腺体受累的严重程度进行个体化治疗。GH 分泌腺瘤和 ACTH 分泌腺瘤需要积极治疗，而无功能垂体腺瘤可以密切观察。
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