Long-Term Natural Course of Pituitary Tumors in Patients With MEN1: Results From the DutchMEN1 Study Group (DMSG)

Abstract

Systematic presymptomatic screening for PIT in patients with MEN1 predominantly results in detection of nonfunctioning microadenomas. Prolactinoma in patients with MEN1 responded well to medical treatment. Microadenomas grew only occasionally and after many years without clinical consequences. Frequent magnetic resonance imaging followup of nonfunctioning microadenomas in the context of MEN1 and sporadically occurring PITs therefore seems debatable.

The genetic background of acromegaly

“…MEN1: 40% of MEN1 gene mutation carriers have a pituitary adenoma and 25% of these have acromegaly, with very few cases described in children [18, 19]. We note, however, that in a systematic screening study [10] a large number of small NFPAs have been identified and the percentage of acromegaly is only 7% among the pituitary adenoma patients in this study. GHRH-secreting pancreas tumors can also lead to acomegaly or gigantism in MEN1 patients.…”

“…Familial isolated pituitary adenoma syndrome is the most common familial cause of acromegaly/gigantism, as available data suggest that syndromic familial acromegaly, due to Carney complex, MEN1, MEN4 and SDH-related syndromes, are less common [6–10]. …”

“…Pituitary tumors occur in 38% (15–50%) of the cases presenting with pituitary disease [10, 53, 54], with prolactinomas being the most frequent (60%) and somatotropinomas being the second most common (25%) (Fig. 1) [19].…”

“…Interestingly, a prospective screening study found different proportions of pituitary disease subtypes with significantly higher number non-functioning adenomas. These differences might be explained by the different strategies used to collect the data (spontaneous presentation vs. clinical screening) [10] and it is unclear what percentage of these often small non-functioning adenomas represent, in fact, incidentalomas. Pituitary tumors in MEN1 occur more frequently in women, prolactinomas are more common in younger patients and are often invasive macroadenomas.…”

Multiple endocrine neoplasia syndrome type 1: institution, management, and data analysis of a nationwide multicenter patient database

“…The collection of clinical, biochemical, and genetic characteristics of unselected patients with rare disorders, such as MEN1, in multicentre-nationwide registries or databases, is a useful approach to increase knowledge of epidemiological aspects of the disease and the natural course and prognosis of single manifestations of the syndrome [16][17][18][19][20].…”