Multiple Endocrine Neoplasia Type 1

Multiple endocrine neoplasia: an update

“…It is this cohort that is often missed in follow up, as the relatively high incidence of MEN1 is not well known. In pituitary tumours, the incidence is rarer (<3%), but in certain pancreatic pathologies, such as gastrinomas, the incidence of MEN1 reaches 16–38% . Hence, in these groups, the pretest probability of MEN1 is high, and genetic testing should be considered (Box 2).…”

“…The most common clinical feature of MEN1 is primary hyperparathyroidism usually related to parathyroid hyperplasia (although occasionally dominant adenomas develop), affecting 95% of patients . Hypercalcaemia is the most common initial manifestation .…”

“…It may be asymptomatic, or less commonly, it can produce signs and symptoms, including lethargy, depression, constipation, polyuria, polydipsia, renal stones and osteoporosis. Hyperparathyroidism associated with MEN1 tends to have an earlier age of onset than sporadic hyperparathyroidism (by approximately 20 years), and an even gender distribution …”

PET/CT imaging of neuroendocrine tumors with 68Gallium-labeled somatostatin analogues: An overview and single institutional experience from India

“…They are usually functional and commonly include gastrinomas (60%) and insulinomas (10%), although carcinoid tumors are also known to occur. [80] MEN2 syndrome on other hand is associated with MTC and pheochromocytoma. [81] As most of these tumors express SSTRs, 68 Ga-DOTA-peptide PET/CT can play an important role in management of these disorders.…”

Multiple endocrine neoplasia type 1

“…[1] MEN1 is inherited as an autosomal-dominant disorder with a high degree of penetrance, such that > 95% of patients develop clinical manifestations of the disorder by the fifth decade. [2] The earliest age at which manifestations of MEN1 may occur has been reported to be five years. [3] Parathyroid tumors, which lead to hypercalcemia, are the most common feature of MEN1 and occur in about 95% of patients.…”