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Endocrine > Adrenal Histology > Flashcards

Adrenal Histology Flashcards

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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

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A

paroxysmal hypertension, headache, diapheresis

20
Q

T/F pheochromocytoma is rare in children

21
Q

Genes associated with familial pheochromocytoma

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A

VHL,RET, NF1, SDHB,SDHD

22
Q

Rule of ten

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A

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

23
Q

Familial pheochromocytomas are uni/multicentric

24
Q

Most malignant familial pheochromocytomas are of what gene type?

25
__________ lesions of pheochromocytoma or association with medullary hyperplasia suggest genetically linked syndrome and hence lesions in other organs.
bilaterality/multifocal --> especially true if pt <40 //must consider family disease
26
Pheochromocytoma malignancy is only defined by
metastasis
27
T/F necrosis, mitoses, and vascular invasion indicate malignancy of pheochromocytoma
F
28
MEN Syndromes
genetically defined and involve multiple endocrine organs --> may also have lesions in non endocrine systems
29
3 main MENS
MEN 1, 2A, 2B
30
Werner syndrome
MEN1: multifocal hyperplasia and neoplasia of Pancreatic islets, Pituitary, Parathyroid (also adrenal cortex, lung, thymus) --> 3Ps
31
Sipple syndrome
MEN 2A: neoplasia and hyperplasia of thyroid C cells, adrenal medulla, and parathyroids
32
Gene involved in MEN1
menin
33
Clinical prognosis of MEN1 is related to
pancreatic lesions --> usually malignant
34
MEN 2A and 2B involve mutations in ___
ret protooncogene
35
Most prognostically important lesion in MEN 2A/B
medullary thyroid carcinoma
36
MEN 2B
neoplasia and hyperplasia of thyroid C cells and adrenal AND neural tissue of oral/GI, skeletal/eye lens abnormalities
37
MEN Syndromes
genetically defined and involve multiple endocrine organs --> may also have lesions in non endocrine systems
38
3 main MENS
MEN 1, 2A, 2B
39
Werner syndrome
MEN1: multifocal hyperplasia and neoplasia of Pancreatic islets, Pituitary, Parathyroid (also adrenal cortex, lung, thymus) --> 3Ps
40
Sipple syndrome
MEN 2A: neoplasia and hyperplasia of thyroid C cells, adrenal medulla, and parathyroids
41
Gene involved in MEN1
menin
42
Clinical prognosis of MEN1 is related to
pancreatic lesions --> usually malignant
43
MEN 2A and 2B involve mutations in ___
ret protooncogene
44
Most prognostically important lesion in MEN 2A/B
medullary thyroid carcinoma
45
MEN 2B
neoplasia and hyperplasia of thyroid C cells and adrenal AND neural tissue of oral/GI, skeletal/eye lens abnormalities
46
96% of MEN 2B have mutation in codon ___ of ret
918 (intracellular part of ret)

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