Pituitary adenomas in the setting of multiple endocrine neoplasia type 1: a single-institution experience

被引:14
作者
Cohen-Cohen, Salomon [1 ]
Brown, Desmond A. [1 ]
Himes, Benjamin T. [1 ]
Wheeler, Lydia P. [1 ]
Ruff, Michael W. [2 ]
Major, Brittny T. [3 ]
Ospina, Naykky M. Singh [4 ]
Atkinson, John L. D. [1 ]
Meyer, Fredric B. [1 ]
Bancos, Irina [4 ]
Young Jr, William F. [4 ]
Van Gompel, Jamie J. [1 ,5 ]
机构
[1] Mayo Clin, Dept Neurol Surg, Rochester, MN USA
[2] Mayo Clin, Dept Neurol, Rochester, MN USA
[3] Mayo Clin, Dept Biostatist, Rochester, MN USA
[4] Mayo Clin, Dept Endocrinol, Rochester, MN USA
[5] Mayo Clin, Dept Otolaryngol Head & Neck Surg, Rochester, MN USA
关键词
MEN1; pituitary adenomas; asymptomatic; transsphenoidal surgery; pituitary surgery; PROLACTINOMAS; MEN1; DISEASE;
D O I
10.3171/2020.1.JNS193538
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
OBJECTIVE Multiple endocrine neoplasia type 1 (MEN1) is a rare, autosomal-dominant tumor disorder characterized by the development of pituitary tumors and other endocrine neoplasms. Diagnosis is made clinically based on the development of 2 or more canonical lesions (parathyroid gland, anterior pituitary, and enteropancreatic tumors) or in family members of a patient with a clinical diagnosis of MEN1 and the occurrence of one of the MEN1-associated tumors. The goal of this study was to characterize pituitary tumors arising in the setting of MEN1 at a single institution. The probability of tumor progression and the likelihood of surgical intervention in patients with asymptomatic nonfunctional pituitary adenomas were also analyzed. METHODS A retrospective review of a prospectively maintained institutional database was performed for patients with MEN1 diagnosed from 1970 to 2017. Data included patient demographics, tumor characteristics, treatment strategies, and outcomes. RESULTS A review of the database identified 268 patients diagnosed with MEN1, of whom 158 (59%) were female. Among the 268 patients, 139 (51.8%) had pituitary adenomas. There was a higher prevalence in women than in men (65% vs 35%, p < 0.005). Functional adenomas (57%) were more common. Prolactin-secreting adenomas were the most common functional tumors. Macroadenomas were seen in 27% of patients and were more likely to be symptomatic and locally aggressive (p < 0.001). Forty-nine patients (35%) underwent transsphenoidal resection at some point during their disease course. In 52 patients who were initially observed with MEN1 asymptomatic nonfunctional adenomas, only 5 (10%) progressed to need surgery. In MEN1 patients, an initial parathyroid lesion is most likely followed in order by pitu- itary, pancreatic, adrenal, and, finally, rare carcinoid tumors. CONCLUSIONS Asymptomatic nonfunctional pituitary adenomas in patients with MEN1 may be followed safely with MRI. In this series, parathyroid tumors developed at the lowest median age of all cardinal tumors, and development of additional cardinal MEN1 lesions followed a predictable pattern. This pattern of disease progression could have signifi- cant implications for disease surveillance in clinical practice and may help to target clinical resources to the lesions most likely to develop next. This may aid with early detection and treatment and warrants further study.
引用
收藏
页码:1132 / 1138
页数:7
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