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Endocrine - MSI Spring > MEN > Flashcards

MEN Flashcards

1
Q

How is MEN inherited?

A

as an autosomal dominant trait with a high degree of penetrance and variable expressivity

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2
Q

What are the major forms of MEN syndromes?

A

Type 1, type 2 (2A and 2B) and type 4

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3
Q

What is the prevalence of MEN 1

A

Estimated to vary btwn 2-20 per 100,000

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4
Q

What is the prevalence of MEN 2

A

Less common than MEN 1
2A most common form of type 2
2B: relatively uncommon, 5% of all type 2 cases

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5
Q

Where do MEN tumors arise from?

A

Amine precursor uptake and decarboxylation (APUD) group of cells, they constitute the diffuse system of neuroendocrine cells distributed throughout the body

Inherited genetic defects affect different groups of APUD cells and lead to neoplastic development of related cell types in diverse anatomic regions

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6
Q

What do APUD cells do?

A

synthesize and/or secrete biogenic amines formed through activity of L-dopa decarboxylase

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7
Q

What are the common features of APUD cells?

A 
Biogenic amine synthesis 
Amine precursor uptake
Amine (DOPA) decarboxylase
Small polypeptide hormone synthesis
Membrane-bound neurosecretory granules
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8
Q

What kind of tumors will exhibit in MEN 1

A 
Parathyroid adenoma ~95%
Pancreatic endocrine tumor ~50%
Pituitary adenoma
Carcinoid tumor
Functional and non-functional adrenal cortical hyperplasia or adenomas
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9
Q

What kind of tumors will exhibit in MEN 2A?

A

Medullary thyroid carcinoma ~ 100%
Bilateral phaeochromocytoma ~50%
Parathyroid adenoma

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10
Q

What kind of tumors will exhibit in MEN 2B?

A

Neuromas ~ 100%
Medullary carcinoma of the thyroid ~ 100%
Marfanoid habitus ~ 100%
Phaeochromocytoma ~50%

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11
Q

What are the conditions associated with MEN 1

A 
Hyperparathyroidism
Hypercalcemia
Zollinger-Ellison syndrome
Acromegaly of pituitary: Tumor-secreting GH
Cushing: tumor secreting ACTH
Galactorrhea
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12
Q

What are the conditions associated with MEN IIa?

A

Hyperparathyroidism
Hypercalcemia
Increased calcitonin
Increased catecholamines

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13
Q

What are the conditions associated with MEN IIb?

A

Mucosal nodules
Marfanoid body habitus
Increased calcitonin
increased catecholamines

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14
Q

What is MEN type I caused by?

A

mutations in MEN 1 gene, which encodes a protein known as menin: tumor suppressor

Mutations in menin causes unregulated cell division that leads to tumor formation

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15
Q

Describe the different manifestations of MEN 1 that can occur between male vs female

A

Male: predilection for developing carcinoid tumors within the thymus
Female: bronchial carcinoids

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16
Q

What are the three means of diagnosing MEN 1?

A

Clinical: two or more MEN 1- associated tumors
Familial: pt with 1 MEN 1 associated tumor and a first-degree relative with MEN 1
Genetic: asymptomatic carrier of MEN 1 mutation

17
Q

In MEN 1, what is the most frequently involved organ?

A

Parathyroid glands and hyperparathyroidism is the first manifestation
Hyperplasia of all four glands

18
Q

When is the presence of MEN 1 detected?

A

disease of the pituitary or pancreas has brought the pt to medical attention

19
Q

What is the second most common manifestation of MEN 1?

A

neoplastic transformation of the pancreatic islet cells: Benign or malignant
Most frequent manifestation of pancreatic involvement is gastrinoma (1/3)

20
Q

What are the second most common type of functional pancreatic tumor in MEN 1?

A

insulinomas

*others include: glucagonomas, somatostatinomas, VIPomas

21
Q

Describe most pituitary tumors found in MEN 1

A

Functionally active and secrete prolactin
Symptoms: hyperprolactinemia, galactorrhea and amenorrhea in women
Impotence in men
Symptoms are 3rd most common manifestations of MEN 1

22
Q

What is the Tx for MEN 1?

A

Surgical resection of hyperplastic parathyroid tissue
Surgical resection of pituitary adenomas or pharmacological management with bromocriptine (prolactinomas) and octreotide (acromegaly)
Subtotal pancreatectomy - not usually possible

23
Q

What mutation causes MEN type 2?

A

RET protooncogene: gene codes for a receptor tyrosine kinase that phosphorylates and activates enzymes critical to cellular development

24
Q

What is the most common sigh of MEN 2?

A

malignant transformation of parafollicular cells or C cells = medullary thyroid carcinoma (MTC)

25
Q

Which MEN type 2 is more severe?

A

MEN 2B - 30 years avg age

MEN2A- 60

26
Q

What is another name for MEN 1?

A

Wermer syndrome

27
Q

What is another name for MEN 2A?

A

Sipple syndrome

28
Q

What does MEN2A consists of?

A

neoplastic transformation of parathyroids, thyroid parafollicular C cells and adrenal medulla

29
Q

Parafollicular cells in pts with MEN 2A characteristically progress through a state of C-cell hyperplasia to what over time?

A

nodular hyperplasia to malignant degeneration over a variable period

30
Q

Medullary thyroid carcinoma (MTC) expreses peptides and hormones not commonly elaborated by ___ cells, including what?

A

C

Somatostatin, TRH, VIP, POMC, carcinoembryonic antigen, and neurotensis

31
Q

Pheochromocytomas with MEN 2A secrete greater amounts of _______ than sporadic phenochromatcytomas

A

epinephrine

32
Q

What is cutaneous lichen amyloidosis?

A

an itchy skin condition, might be present in some cases of MEN 2A

33
Q

hyperparathyroidism (is/is not) associated with MEN 2B

A

is not

34
Q

What type of MEN?

MTC, pheochromocytoma, primary hyperparathyrroidism, cutaneous lichen amyloidosis, Hirschprung?

A

2A

35
Q

What type of MEN?

MTC, pheochromocytoma, mucosal neuromas, intestinal ganglioneuromas, marfanoid habitus, NO hyperparathryoidism

A

2B

36
Q

What are the symptoms of gastrinomas associated with MEN 1

A

Prolific production of gastric acid with resultant duodenal and jejunal ulcers and diarrhea

37
Q

What do MEN syndromes typically involve?

A

Tumors (neoplasia) in at least two endocrine glands

Tumors can be noncancerous (benign) or malignant

Endocrine - MSI Spring (15 decks)

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