20 - Adrenal and Thymus path

Question 1

what is made in adrenal medulla

Answer 1

catecholamines

Question 2

layers of adrenal cortex from outer to inner

Answer 2

GFR - glomerulosa, fasciculata, reticularis

Question 3

stress response and adrenal gland path

Answer 3

acute stress in adults - lipid depletion in adrenal cortex
chronic stress - adrenocortical hyperplasia

acute stress in neonates - lipid accumulation in cortex and pseudoglandular change in zona glomerulosa

Question 4

MC source of ectopic ACTH

Answer 4

small cell lung tumor

Question 5

MC cause of primary hyperaldosteronism

Answer 5

Conn syndrome (adenoma making aldosterone)

Question 6

congenital adrenal hyperplasia - inheritance/mutation, pathophys

Answer 6

AR
usually def or absence of 21-hydroxylase (CYP21B gene defect)
> dec cortisol +/- aldosterone, excess androgens (alt means of synthesis) > inc ACTH via feedback > adrenocortical hyperplasia

Question 7

congenital adrenal hyperplasia - clinical features

Answer 7

variable severity
androgen excess - ambiguous genitalia at birth (esp females), precocious puberty in males
variable aldosterone def - can have salt wasting

Question 8

Waterhouse-Friderichsen syndrome

Answer 8

bacteremia (most often n. meningitidis but can be pseudomonas, s pneumo, h flu, or staph) causes massive adrenal hemorrhage > adrenal crisis

Question 9

autoimmune polyendocrinopathy syndrome type 1

Answer 9

chronic mucocutaneous candidiasis, skin/nail/enamel abnl, hypoadrenalism, hypoPTH, hypogonadism, pernicious anemia

AIRE gene

Question 10

autoimmune polyendocrinopathy syndrome type 2

Answer 10

adrenal insufficiency, autoimmune thyroiditis, T1DM

Question 11

Beckwith-Wiedemann syndrome

Answer 11

syndrome assoc w/ adrenocortical CA

Question 12

pheo presentation

Answer 12

paroxysmal BP elevation w/ tachycardia, HA, sweating, tremors, apprehension

Question 13

pheo rule of 10s

Answer 13

10% familial syndrome (MEN2a,2b, NF1, VHL, SW)
10% extraadrenal
10% bilat
10% malignant behavior
10% childhood

Question 14

MEN1

Answer 14

parathyroid
pancreas
pituitary
(also gastrinoma in duodenum, carcinoid tumors, thyroid and adrenal adenomas, lipomas)

Question 15

MEN2a

Answer 15

medullary thyroid CA (100%)
pheo
PTH hyperplasia

Question 16

MEN2b

Answer 16

medullary thyroid CA
pheo
mucosal neuromas
marfanoid features

Question 17

MC extracranial solid tumor of childhood

Answer 17

neuoblastic tumors

Question 18

when is thymus at max size?

Answer 18

puberty

Question 19

22q11 deletion syndromes mnemonic

Answer 19

CATCH22

cardiac, abnl facies, thymic hypoplasia, cleft palate, hypoCa

Question 20

follicular thymic hyperplasia assoc w/ what other condition

Answer 20

myasthenia gravis

Question 21

paraneoplastic phenomena of thymoma

Answer 21

acquired hypogammaglobulinemia, pure red cell aplasia, Grave’s, pernicious anemia, cushing

Question 22

path appearance of thymoma

Answer 22

lobulated firm gray/white mass +/- necrosis and calcification
micro - lobules w/ fibrous bands
epithelial cells w/ variable lymphs

Question 23

two types of malignant thymomas

Answer 23

type 1 - benign cytology, malignant by local invasion only

type 2 - malignant cytology, very aggressive