Multiple Endocrine Neoplasia Flashcards
types of MEN from most prevalent to least
MEN1, MEN2A, MEN2B
what kind of cells do MEN tumors arise from and what do they do
APUD: can syntheize and or secrete biogenic amines formed through actiity of L-DOPA decarboxylase
what is a carcinoid
slow growing type of neuroendocrine tumor
what is a carcinoma
cancer related to epithelial cells
what is an adenoma
benign, epithelial origin associated with a gland
what is a neuroma
tumor in nerve tissue
MEN 1 has what tumors (in order of prevelance)
parathyroid adenoma (95%), pancreatic endocrine tumors (50%), pituitary adenoma (40%), carcinoid tumors
MEN 2A tumors
medullary thyroid carcinoma, bilateral pheochromocytomas, parathyroid adenoma
MEN 2B tumors
medullary thyroid carcinoma, neuromas, phaeochromocytoma
mucosal neuromas and marfanoid habitus occur in what disease
MEN 2B
MEN I associations
hyperparathyroidism–>hypercalcemia
ZE syndrome
pituitary path: Galactorrhea, Acromegaly, Cushing
MEN IIA associations
hyperparathyroidism hypercalcemia medullary carcinoma increased calcitonin increased catecholamines
MEN IIB associations
mucosal nodules marfanoid body habitus medullary carcinoma increased calcitonin increased catecholamines
MEN 1 is caused by mutations in ____ gene which encodes a protein known as ___ which is a ____
MEN1 gene, menin, tumor suppressor
MEN1: male patients appear to have predilection for developing ____ tumors
women for developing ____ carcinoids
carcinoid tumors in thymus for men
bronchial carcinoids in women
diagnosins MEN 1
clincial
failial
genetic
clinical: 2 or more MEN tumors
familial: 1 MEN tumor and first degree relative with MEN 1
genetic: asymptomatic carrier of MEN1 mutation
the most frequent manifestation of MEN 1 pancreatic involvement is what
gastrinoma, then insulinomas
also have glucagonomas, somatostatinomas, VIPomas
pituitary tumors of MEN1
-most secrete what and causes what
most secrete prolactin and have hyperprolactinemia (galactorrhea and amenorrhea in women, impotence in men)
MEN1 patients may develop tumor secreting __ or __ as well
ACTH and GH
treatment for MEN1
surgery
bromocriptine: dopamine agonist, inhibits prolactin
octreotide: somatostatin analogue, inhibits GH secretion by AP
- subtotal pancreatectomy (remove as much tumor as possible)
MEN 2 is caused by a mutation in what gene
RET protooncogene that codes for receptor TK
what is the most common sign of MEN 2
malignant transformation of parafollicular cells
MEN 2A or sipple syndrome parafollicular cell progression
C cell hyperplasia to nodular hyperplasia to malignant degeneration over a variable period
hyperparathyroidism is associated with MEN2A and similar to that of MEN-1 but is ____ frequent than MEN1
less
