Pituitary Tumors

In MEN 1 kindreds, pituitary tumors are found less frequently than primary HPT or PETs.40 Signs and symptoms related to a pituitary adenoma are the initial clinical presentation of MEN 1 in up to 25% of "first in the kindred" cases but in less than 10% of familial cases that are

TABLE 76-2. Indications for Genetic Screening

At-risk family members in known MEN t kindreds Young patients (<50 years) with multigland or recurrent

HPT (normal renal function) Patients with prolactinomas {14% MEN 1) Patient with multiple PETs

Patients with any MEN 1 iesion and an adrenal lesion Patients with bronchial or thymic carcinoids

MEN 1 = multiple endocrine neoplasia type 1; PET = pancreatic endocrine tumor; HPT = hyperparathyroidism.

diagnosed prospectively.53 The estimated prevalence of pituitary tumors in MEN 1 patients ranges from 10% to 60%.5355 For example, in a series of 324 patients with MEN 1, pituitary adenomas occurred in 197 (42%); mean age at diagnosis was 38 years (range, 12 to 83 years).56 In most MEN 1 patients, the pituitary tumors are macroadenomas (>10 mm) (Fig. 76-1).56 All subtypes of pituitary adenoma have been reported in MEN 1, but most frequently they are prolactinomas (-60%). Patients may present with amenorrhea/galact-orrhea in women, symptoms related to hypogonadism in men, and/or sellar mass effect symptoms.53,56,57 Less common pituitary tumor subtypes in MEN 1 include somatotroph adenoma (acromegaly; «25%), corticotroph adenoma (Cushing's syndrome; =5%), and clinically nonfunctioning pituitary tumors (=10%). Monitoring for pituitary tumor development in the MEN 1 patient should include measurement of serum prolactin and insulin-like growth factor 1 as well as imaging of the pituitary by magnetic resonance imaging (MRI) every 2 to 3 years.30 In patients with an abnormal pituitary MRI, hypothalamic-pituitary testing should be completed to characterize the type of the pituitary adenoma and its effects on the secretion of other pituitary hormones. Treatment of pituitary tumors in MEN 1 is guided by the adenoma subtype and is identical to that in patients with sporadic pituitary tumors (Fig. 76-2). In addition, after successful pharmacologic or surgical treatment of the pituitary tumor, MEN 1 patients should continue to be evaluated with periodic pituitary tumor screening since a second pituitary adenoma may arise from the remaining pituitary tissue.

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