We briefly
review the characteristics of
pituitary tumors associated with
multiple endocrine neoplasia type 1.
Multiple endocrine neoplasia type 1 is an autosomal-dominant disorder most commonly characterized by
tumors of the pituitary, parathyroid, endocrine-
gastrointestinal tract, and
pancreas. A
MEDLINE search for all available
publications regarding
multiple endocrine neoplasia type 1 and
pituitary adenomas was undertaken. The
prevalence of
pituitary tumors in
multiple endocrine neoplasia type 1 may vary from 10% to 60% depending on the studied series, and such
tumors may occur as the first clinical manifestation of
multiple endocrine neoplasia type 1 in 25% of sporadic and 10% of familial cases.
Patients were younger and the
time between initial and subsequent
multiple endocrine neoplasia type 1 endocrine lesions was significantly longer when
pituitary disease was the initial manifestation of
multiple endocrine neoplasia type 1.
Tumors were larger and more invasive and clinical manifestations related to the size of the
pituitary adenoma were significantly more frequent in
patients with
multiple endocrine neoplasia type 1 than in subjects with non-
multiple endocrine neoplasia type 1. Normalization of pituitary hypersecretion was much less frequent in
patients with
multiple endocrine neoplasia type 1 than in subjects with non-
multiple endocrine neoplasia type 1.
Pituitary tumors in
patients with
multiple endocrine neoplasia type 1 syndrome tend to be larger, invasive and more symptomatic, and they tend to occur in younger
patients when they are the initial presentation of
multiple endocrine neoplasia type 1.