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Endo/Repro Midterm > Multiple Endocrine Neoplasia > Flashcards

Multiple Endocrine Neoplasia Flashcards

1
Q

Multiple endocrine neoplasia typically involves ____ endocrine glands and tumors may be benign or malignant

What are the hallmarks of malignancy?

A

Two

Metastasis; invasion

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2
Q

Genetic inheritence of Multiple Endocrine Neoplasia

A

Autosomal dominant trait (with high penetrance and variable expression)

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3
Q

What is the most common type of MEN?

A

MEN 1 (followed by MEN 2A, then 2B)

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4
Q

How can so many various endocrine organs be affected in MEN syndromes?

A

Because they arise from the same type of cells - amine precursor uptake and decarboxylation (APUD) group of cells

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5
Q

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

A

Neuroendocrine; amines; L-dopa decarboxylase

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6
Q

Hyperplasia vs. hypertrophy

A

Hyperplasia = increased number of cells

Hypertrophy = increased size of cells

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7
Q

Abnormal mass of tissue; growth exceeds and is uncoordinated with that of normal tissues; persists after cessation of stimuli

A

Neoplasm

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8
Q

MEN 1 is associated with what other endocrine condition 95% of the time?

A

Hyperparathyroidism

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9
Q

MEN 2A and B often occur in combination with what type of tumor?

A

Medullary thyroid carcinoma

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10
Q

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)

A

Mucosal neuromas

Marfanoid habitus

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11
Q

Pituitary adenoma is typically associated with what type of MEN?

A

MEN 1

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12
Q

40-50% of tumors associated with MEN 2A and B are _____

A

Pheochromocytomas

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13
Q

An enteropancreatic tumor would most likely be associated with what type of MEN?

A

MEN 1

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14
Q

MEN1 is also known as _____ syndrome

A

Wermer

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15
Q

MEN 1 involves a gene mutation encoding the ____ protein, which typically functions as a _____________, so mutations cause unregulated cell division and thus tumor formation

A

Menin; tumor suppressor

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16
Q

3 P’s of Men 1

A

Parathyroid
Pancreas (endocrine)
Pituitary

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17
Q

______ tumors are frequently reported in MEN 1 patients, which males have a predilection for in the ________, whereas _____ type occurs almost exclusively in women

A

Carcinoid; thymus; bronchial

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18
Q

PPM of MEN 2A

A

Pheochromocytoma
Parathyroid (hypercalcemia)
Medullary thyroid cancer

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19
Q

PMM of MEN 2B

A

Pheochromocytoma
Mucocutaneous tumors
Medullary thyroid cancer

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20
Q

What are the 3 means of diagnosing MEN 1?

A

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)

21
Q

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

A

Hyperparathyroidism; pituitary; pancreas

22
Q

Hyperparathyroidism associated with MEN 1 results in hyperplasia in ___/4 parathyroid glands

A

4

23
Q

Second most common manifestation of MEN 1

A

Neoplastic transformation of pancreatic islet cells (may be benign or malignant)

24
Q

Neoplastic transformation of pancreatic islet cells is the second most common manifestation of MEN 1.

Of these, what are the 2 most frequent types?

A

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]

25
Q

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

A

Prolactin; galactorrhea; amenorrhea; impotence

26
Q

MEN 1 patients may develop tumors secreting ___ (Cushing syndrome) or ______(acromegaly)

A

ACTH; GH

27
Q

T/F: MEN 1 pts have an increased frequency of both functional and nonfunctional cortical hyperplasia or adenomas

A

True

28
Q

Tx options for MEN 1

A

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)

29
Q

Drug tx for prolactinoma-associated MEN1

A

Bromocriptine

30
Q

Drug tx for acromegaly-associated MEN1

A

Octreotide

31
Q

MEN 2 is caused by an activation mutation of the ____ protoconcogene, which codes for a RTK that phosphorylates and activates enzymes critical to cellular development

A

RET

32
Q

What is the most common sign of MEN2?

A

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

33
Q

Medullary thyroid carcinoma is the most common sign of MEN 2, meaning malignant transformation of the C cells has occurred. What are the normal functions of C cells?

A

Secretion of calcitonin (normally scattered throughout the thyroid gland)

34
Q

MEN 2 is divided into type 2A and type 2B. Which type is associated with more severe mortality overall?

A

MEN2B

[avg death for 2A = 60y/o, avg death for 2B = 30y/o]

35
Q

T/F: medullary thyroid cancer is often bi-lobed

A

True

36
Q

Neoplastic C-cell hyperplasia involves expanding C-cell masses disrupting the the _____ membrane surrounding individual ______ follicles

A

Basal; thyroid

37
Q

MEN2A consists of neoplastic transformations of what 3 major structures? This is also known as what syndrome?

A

Parathyroids
Thyroid parafollicular C cells
Adrenal medulla

Sipple syndrome

38
Q

_______ cells in patients with MEN 2A characteristically progress through a state of ______ cell hyperplasia to nodular hyperplasia to malignant degeneration over a variable period

A

Parafollicular; C-cell

39
Q

Medullary thyroid cancer associated with MEN2A Sipple syndrome also expresses peptides and hormones NOT commonly elaborated by C-cells - what are some examples?

A 
Somatostatin
TRH
VIP
POMC
Carcinoembryonic antigen (CEA)
Neurotensin
40
Q

Pheochromocytomas associated with MEN 2A secrete greater amounts of _____ than sporadic pheochromocytomas

Another condition called ______ may be present, characterized by pruritic, scaly, papular, pigmented skin lesion located in interscapular region or on extensor surfaces of extremities. Associated _____ deposition has been documented histologically

A

Epinephrine

Cutaneous lichen amyloidosis; amyloid

41
Q

Is it more frequent to find hyperparathyroidism in MEN 1 or MEN 2A?

A

MEN 1

[although the type of hyperparathyroidism and its manifestations are similar]

42
Q

What makes up the MEN 1 “Diamond” diagram?

A

Pituitary
Parathyroid — Parathyroid
Pancreas

43
Q

What makes up the MEN 2A “Square” diagram?

A

Parathyroid — Parathryoid

Pheo (adrenals) — Pheo (adrenals)

44
Q

What makes up the MEN 2B “Triangle” diagram?

A

Oral

Pheo (adrenals) — Pheo (adrenals)

45
Q

MEN 2B is associated with medullary thyroid cancer, pheochromocytoma, and younger age of death.

What are the other 3 identifying characteristics of MEN 2B?

A

Mucosal neuromas
Intestinal ganglioneuromas (similar to hirschprung presentation)
Marfanoid body habitus

46
Q

Describe marfanoid body habitus associated with MEN 2B

A

Long limbs
Arachnodactyly
Hyperlaxity

47
Q

T/F: hyperparathyroidism is often associated with MEN 2B

A

False; not associated

48
Q

T/F: the MTC associated with MEN 2B is more aggressive than other forms with metatatic lesions as early as infancy

A

True

Endo/Repro Midterm (14 decks)

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