Adrenal Histology Flashcards

1
Q

causes of hypercortisolism

A

bilateral adrenal hyperplasia (pituitiary acth, ectopic acth, primary, idiopathic), adenoma, carcinoma

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2
Q

_____ cancer is the most important cause of ectopic acth production

A

small cell lung cancer

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3
Q

gross features of adrenal corticocarcinoma

A

large, necrosis, hemorrhage, invasion

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4
Q

poor prognostic factors for adrenal corticocarcinoma

A

mitotic activity, venous invasion, p53+, Ki67+, large size

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5
Q

Weiss scoring

A

tumors with a score > 3 are malignant (scored based on histological features)

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6
Q

LiFraumeni

A

p53

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7
Q

Beckwith-Wiedemann

A

11p15 –> Wilm’s tumor

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8
Q

familial adenomatous polyposis coli

A

APC

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9
Q

Multiple endocrine neoplasia type 1

A

Menin (11q13)

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10
Q

An adenoma that is REALLY big is probably a ___

A

tumor of uncertain malignant potential : UMP

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11
Q

Female/Male: simple diffuse adrenal hyperplasia

A

females

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12
Q

Female/Male: bilateral nodular hyperplasia

A

females

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13
Q

Female/Male: ectopic adrenal hyperplasia

A

males

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14
Q

Primary cause of hypercortisolism in adults

A

hyperplasia > adenoma/carcinoma

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15
Q

Primary cause of hypercortisolism in children

A

carcinoma > hyperplasia > adenoma

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16
Q

Causes of hyperaldosteronism

A

adenoma> bilateral hyperplasia // carcinoma RARE

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17
Q

Causes of hypoaldosteronism

A

autoimmune destruction, replacement (tb, fungi, amyloid, tumor)

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18
Q

Causes of adrenal medulla hyperfunction

A

pheochromocytoma, hyperplasia, neuroblastoma

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19
Q

Clinical triad for pheochromocytoma

A

paroxysmal hypertension, headache, diapheresis

20
Q

T/F pheochromocytoma is rare in children

A

T

21
Q

Genes associated with familial pheochromocytoma

A

VHL,RET, NF1, SDHB,SDHD

22
Q

Rule of ten

A

pheochromocytomas: 10% bilateral, 10% extraadrenal, 10% malignant

23
Q

Familial pheochromocytomas are uni/multicentric

A

multicentric

24
Q

Most malignant familial pheochromocytomas are of what gene type?

A

SDHB

25
Q

__________ lesions of pheochromocytoma or association with medullary hyperplasia suggest genetically linked syndrome and hence lesions in other organs.

A

bilaterality/multifocal –> especially true if pt <40 //must consider family disease

26
Q

Pheochromocytoma malignancy is only defined by

A

metastasis

27
Q

T/F necrosis, mitoses, and vascular invasion indicate malignancy of pheochromocytoma

A

F

28
Q

MEN Syndromes

A

genetically defined and involve multiple endocrine organs –> may also have lesions in non endocrine systems

29
Q

3 main MENS

A

MEN 1, 2A, 2B

30
Q

Werner syndrome

A

MEN1: multifocal hyperplasia and neoplasia of Pancreatic islets, Pituitary, Parathyroid (also adrenal cortex, lung, thymus) –> 3Ps

31
Q

Sipple syndrome

A

MEN 2A: neoplasia and hyperplasia of thyroid C cells, adrenal medulla, and parathyroids

32
Q

Gene involved in MEN1

A

menin

33
Q

Clinical prognosis of MEN1 is related to

A

pancreatic lesions –> usually malignant

34
Q

MEN 2A and 2B involve mutations in ___

A

ret protooncogene

35
Q

Most prognostically important lesion in MEN 2A/B

A

medullary thyroid carcinoma

36
Q

MEN 2B

A

neoplasia and hyperplasia of thyroid C cells and adrenal AND neural tissue of oral/GI, skeletal/eye lens abnormalities

37
Q

MEN Syndromes

A

genetically defined and involve multiple endocrine organs –> may also have lesions in non endocrine systems

38
Q

3 main MENS

A

MEN 1, 2A, 2B

39
Q

Werner syndrome

A

MEN1: multifocal hyperplasia and neoplasia of Pancreatic islets, Pituitary, Parathyroid (also adrenal cortex, lung, thymus) –> 3Ps

40
Q

Sipple syndrome

A

MEN 2A: neoplasia and hyperplasia of thyroid C cells, adrenal medulla, and parathyroids

41
Q

Gene involved in MEN1

A

menin

42
Q

Clinical prognosis of MEN1 is related to

A

pancreatic lesions –> usually malignant

43
Q

MEN 2A and 2B involve mutations in ___

A

ret protooncogene

44
Q

Most prognostically important lesion in MEN 2A/B

A

medullary thyroid carcinoma

45
Q

MEN 2B

A

neoplasia and hyperplasia of thyroid C cells and adrenal AND neural tissue of oral/GI, skeletal/eye lens abnormalities

46
Q

96% of MEN 2B have mutation in codon ___ of ret

A

918 (intracellular part of ret)