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Spectrum of Pituitary Disease in Multiple Endocrine Neoplasia Type 1 (MEN 1): Clinical, Biochemical, and Radiological Features of Pituitary Disease in a Large MEN 1 Kindred
journal contribution
posted on 2023-05-16, 10:08 authored by John BurgessJohn Burgess, Shepherd, JJ, Parameswaran, V, Hoffman, L, Timothy GreenawayTimothy GreenawayProlactinomas and somatotropinomas are reported to be the pituitary lesions most frequently associated with multiple endocrine neoplasia type 1 (MEN 1). However, few reports have documented the full spectrum of pituitary disease in this condition. We report herein the clinical, biochemical (PRL, α-subunit, insulin-like growth factor-I, cortisol, and thyroid function), and radiological (magnetic resonance imaging and computerized tomography scan) characteristics of pituitary disease occurring in a single MEN 1 pedigree containing 165 MEN 1-affected members. Pituitary lesions were detected in 30 (18%) of 165 patients overall. In the subgroup of MEN 1 patients (n = 131) living after recognition of MEN 1 in the kindred, pituitary lesions were detected in 25 (19%). In 76% of patients with pituitary lesions, the diagnosis was made by prospective screening; the remainder sought medical attention for symptomatic pituitary disease. Prolactinomas accounted for 76%, and nonfunctioning adenomas accounted for the remaining 24%. α-Subunit elevation was observed in 29% of 41 patients tested, and an aggressive α-subunit secreting macroadenoma developed in 1 subject with a previously documented prolactinoma. Progression of pituitary disease occurred in 47% of patients with prolactinoma. There were no cases of Cushing's disease, thyrotropinoma, or somatotropinoma. We conclude that 1) in addition to prolactinomas, nonfunctioning pituitary tumors are common in MEN 1; 2) α-subunit hypersecretion is frequent in MEN 1; 3) comprehensive screening may identify many clinically significant but asymptomatic pituitary lesions; and 4) prolactinomas occurring in MEN 1 may behave more aggressively than sporadic prolactinomas.
History
Publication title
Journal of Clinical Endocrinology and MetabolismVolume
81Issue
7Pagination
2642-2646ISSN
0021-972XDepartment/School
Tasmanian School of MedicinePublisher
Endocrine SocPlace of publication
Bethesda, Maryland, USARepository Status
- Restricted