MEN Syndromes Flashcards
Tumors seen with MEN usually preceded by what?
asymptomatic stage of endocrine hyperplasia of tissue containing the involved cell-line
Group of genetically inherited dzs resulting in proliferative lesions of multiple endocrine organs
MEN syndromes
MEN 1 is characterized by abnormalities involving which structures?
parathyroid glands, pancreas, and pituitary gland
Most common manifestation of MEN 1
primary hyperparathyroidism
Initial manifestation of MEN 1
hyperparathyroidism
Parathyroid abnormalities seen in MEN 1 include both
hyperplasia and adenomas
leading cause of morbidity and mortality in MEN-1
endocrine tumors of the pancreas
Most common subtypes of endocrine tumors with MEN-1
gastrinomas associated with Zollinger-Ellison, insulinomas associated with hypoglycemia, and neurological manifestations
most frequent anterior pituitary tumor encountered in patients with MEN-1
Prolactinomas
Some men with MEN-1 may develop … from GH secreting tumors
acromegaly
Most common site of gastrinomas in individuals with MEN-1
duodenum
in addition, … (4 tumors) are more frequently noted in MEN 1 pts than in the general population
carcinoid tumors, thyroid and adrenocortical adenomas, lipomas
What are the 3 subsets of MEN-2?
MEN2a, MEN2b, familial medullary thyroid cancer (FMCT)
What is the most common subset of MEN2?
MEN2A
MEN-2A is characterized by the presence or increased risk for development of the what 3 conditions?
medullary thyroid carcinoma, pheochromocytoma, and parathyroid hyperplasia
What occurs in almost 100% of all MEN-2A pts?
medullary carcinoma of the thyroid
MEN-2A related thyroid cancers are usually multifocal and are almost associated with
foci of C-cell hyperplasia in the thyroid tissue adjacent to the carcinoma
thyroid cancers associated with MEN-2A often produce what?
calcitonin
40-50% pts with MEN-2A present with
pheochromocytoma
dusky, enlarged thyroid gland with multiple foci of medullary carcinoma
MEN-2A
… and … occur in 10%-205 of MEN-2A pts
parathyroid hyperplasia and hypercalcemia or renal stones
multiple areas of amyloid deposition
medullary thyroid cancer
Medullary thyroid carcinomas in pts with MEN-2B are usually found to be … and … than the forms of medullary thyroid carcinomas found in MEN-2A pts.
more aggressive, earlier onset
What is not present in patients with MEN-2B
primary hyperparathyroidism
MEN-2B is often accompanied by
neuromas or ganglioneuromas
neuromas or ganglioneuromas in MEN-2B involve which structures?
skin, oral mucosa, eyes, respiratory tract, and GI
both MEN-2A and MEN-2B have an increased risk for the development of what?
intestinal aganglionosis and Hirschsprung dz
