Adrenal Histology Flashcards Preview

Endocrine > Adrenal Histology > Flashcards

Flashcards in Adrenal Histology Deck (46):
1

causes of hypercortisolism

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

2

_____ cancer is the most important cause of ectopic acth production

small cell lung cancer

3

gross features of adrenal corticocarcinoma

large, necrosis, hemorrhage, invasion

4

poor prognostic factors for adrenal corticocarcinoma

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

5

Weiss scoring

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

6

LiFraumeni

p53

7

Beckwith-Wiedemann

11p15 --> Wilm's tumor

8

familial adenomatous polyposis coli

APC

9

Multiple endocrine neoplasia type 1

Menin (11q13)

10

An adenoma that is REALLY big is probably a ___

tumor of uncertain malignant potential : UMP

11

Female/Male: simple diffuse adrenal hyperplasia

females

12

Female/Male: bilateral nodular hyperplasia

females

13

Female/Male: ectopic adrenal hyperplasia

males

14

Primary cause of hypercortisolism in adults

hyperplasia > adenoma/carcinoma

15

Primary cause of hypercortisolism in children

carcinoma > hyperplasia > adenoma

16

Causes of hyperaldosteronism

adenoma> bilateral hyperplasia // carcinoma RARE

17

Causes of hypoaldosteronism

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

18

Causes of adrenal medulla hyperfunction

pheochromocytoma, hyperplasia, neuroblastoma

19

Clinical triad for pheochromocytoma

paroxysmal hypertension, headache, diapheresis

20

T/F pheochromocytoma is rare in children

T

21

Genes associated with familial pheochromocytoma

VHL,RET, NF1, SDHB,SDHD

22

Rule of ten

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

23

Familial pheochromocytomas are uni/multicentric

multicentric

24

Most malignant familial pheochromocytomas are of what gene type?

SDHB

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)