Pituitary Neuroendocrine Tumors in Multiple Endocrine Neoplasia

Pituitary Neuroendocrine Tumors in Multiple Endocrine Neoplasia

Blog Article

Multiple endocrine neoplasia 15-eg2373cl type 1 (MEN1) is an autosomal-dominant disorder characterized by tumors of the pituitary, parathyroid, and endocrine-gastrointestinal tract.Pituitary neuroendocrine tumors (PitNETs) occur in about 40% of MEN1 cases, with 10% being the first manifestation.Recent studies show a slight female predominance, with microPitNETs (1 cm).

Functional PitNETs (FPitNETs) are more frequent than non-functional ones (36% to 48%), with prolactinomas being the most common FPitNETs.MEN1-associated PitNETs are often plurihormonal, larger, and more invasive compared to sporadic types, though simply boho classroom patient age and FPitNET proportions are similar.MEN1 mutation-negative patients tend to have larger, symptomatic PitNETs at diagnosis.

Six patients with MEN1 have been reported to have pituitary carcinomas, including a mutation-negative patient.Treatment approach between PitNETs in MEN1 and sporadic types appears to be similar.PitNETs also occur in MEN4, but their epidemiology is less understood.

In patients with a MEN1-like phenotype and negative genetic testing, MEN4 should be considered.