Flashcards in Multiple Endocrine Neoplasia Deck (48)
Multiple endocrine neoplasia typically involves ____ endocrine glands and tumors may be benign or malignant
What are the hallmarks of malignancy?
Genetic inheritence of Multiple Endocrine Neoplasia
Autosomal dominant trait (with high penetrance and variable expression)
What is the most common type of MEN?
MEN 1 (followed by MEN 2A, then 2B)
How can so many various endocrine organs be affected in MEN syndromes?
Because they arise from the same type of cells - amine precursor uptake and decarboxylation (APUD) group of cells
APUD cells are comprised of ______ cells distributed throughout the body, and have the capacity to synthesize and/or secrete biogenic _____ formed through the activity of ________ enzyme
Neuroendocrine; amines; L-dopa decarboxylase
Hyperplasia vs. hypertrophy
Hyperplasia = increased number of cells
Hypertrophy = increased size of cells
Abnormal mass of tissue; growth exceeds and is uncoordinated with that of normal tissues; persists after cessation of stimuli
MEN 1 is associated with what other endocrine condition 95% of the time?
MEN 2A and B often occur in combination with what type of tumor?
Medullary thyroid carcinoma
About 100% of cases of MEN2B occur in the setting of what other endocrine conditions? (And thus can be used to differentiate 2A and 2B)
Pituitary adenoma is typically associated with what type of MEN?
40-50% of tumors associated with MEN 2A and B are _____
An enteropancreatic tumor would most likely be associated with what type of MEN?
MEN1 is also known as _____ syndrome
MEN 1 involves a gene mutation encoding the ____ protein, which typically functions as a _____________, so mutations cause unregulated cell division and thus tumor formation
Menin; tumor suppressor
3 P’s of Men 1
______ tumors are frequently reported in MEN 1 patients, which males have a predilection for in the ________, whereas _____ type occurs almost exclusively in women
Carcinoid; thymus; bronchial
PPM of MEN 2A
Medullary thyroid cancer
PMM of MEN 2B
Medullary thyroid cancer
What are the 3 means of diagnosing MEN 1?
Clinical: based on presence of 2 or more MEN-1 associated tumors
Familial: patient with one MEN-1 associated tumor + first degree relative with MEN 1 dx
Genetic: asymptomatic carrier of MEN 1 mutation (no biochemical manifestation)
Although the parathyroid gland is the most frequently involved organ in MEN 1, and ______ usually the first manifestation of the syndrome, presence may NOT be detected until clinical diagnosis of ____ or _____ brings the patient to medical attention
Hyperparathyroidism; pituitary; pancreas
Hyperparathyroidism associated with MEN 1 results in hyperplasia in ___/4 parathyroid glands
Second most common manifestation of MEN 1
Neoplastic transformation of pancreatic islet cells (may be benign or malignant)
Neoplastic transformation of pancreatic islet cells is the second most common manifestation of MEN 1.
Of these, what are the 2 most frequent types?
Most frequent = gastrinoma - often ZE syndrome (1/3 of patients with gastrinomas are associated with MEN 1)
Second most = Insulinomas
[other types may include glucagonomas, somatostatinomas, and VIPomas]
42-50% of patients with MEN 1 have pituitary tumors. Most are functionally active and secrete ______; high levels of this hormone are the 3rd most common manifestation of MEN 1, causing ____ and _____in females, and _____ in males
Prolactin; galactorrhea; amenorrhea; impotence
MEN 1 patients may develop tumors secreting ___ (Cushing syndrome) or ______(acromegaly)
T/F: MEN 1 pts have an increased frequency of both functional and nonfunctional cortical hyperplasia or adenomas
Tx options for MEN 1
Surgical resection of hyperplastic parathyroid
surgical resection of pituitary adenomas or pharmacotherapy (bromocriptine for prolactinomas; octreotide for acromegaly)
Subtotal pancreatectomy (surgical cure for associated pancreatic tumors is not usually possible)
Drug tx for prolactinoma-associated MEN1