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
Burgess, JR and Shepherd, JJ and Parameswaran, V and Hoffman, L and Greenaway, TM, 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 of Clinical Endocrinology and Metabolism, 81, (7) pp. 2642-2646. ISSN 0021-972X (1996) [Refereed Article]
Prolactinomas 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.