Pathology of the adrenal glands

Question 1

what are the primary adrenal cortical neoplasms?

Answer 1

cortical adenoma

cortical carcinoma

Question 2

adrenal cortical adenoma: morphology

Answer 2

• well circumscribed, yellow-orange lesions

- may lie within cortex or protrude into medulla or the subscapular region

Question 3

adrenal cortical adenoma: histology

Answer 3

• vacuolated cells due to intracytoplasmic lipid
• mild nuclear pleomorphism
• NO mitotic activity or necrosis

Question 4

adrenal cortical carcinoma: morphology

Answer 4

yellow on cut surface, but usually contains areas of hemorrhage, cystic change, and necrosis

Question 5

adrenal cortical carcinoma: histology

Answer 5

range of well differentiated to markedly anaplastic cells

Question 6

how do adrenal cortical carcinomas typically invade?

Answer 6

vascular channels with metastases to regional lymph nodes and to viscera, especially lung

Question 7

what is anaplasia? what morphological changes is it associated with?

Answer 7

lack of differentiation

• pleomorphism
• abnormal nuclear morphology
• mitoses
• loss of polarity

Question 8

how does adrenal cortical hyperplasia compare with normal adrenal tissue?

Answer 8

adrenal cortex is yellow, thickened, and multinodular

Question 9

what are the three distinctive clinical hyperadrenal syndromes?

Answer 9

• cushing syndrome (excess cortisol)
• hyperaldosteronism (excess mineralocorticoids)
• adrenogenital syndrome (excess androgens)

Question 10

cushing syndrome is caused by _________________

Answer 10

elevation in glucocorticoid levels

Question 11

what are the four causes of cushing syndrome?

Answer 11

pituitary
adrenal
paraneoplastic
iatrogenic (steroid excess)

Question 12

cushing’s disease

Answer 12

excess ACTH coming from the pituitary gland

Question 13

what is the most common cause of primary hyperaldosteronism?

Answer 13

• aldosterone producing adrenocortical neoplasm, usually an adenoma
• primary adrenocortical hyperplasia

Question 14

what is conn syndrome?

Answer 14

a solitary aldosterone secreting adenoma

Question 15

aldosterone producing adenomas (conn syndrome): morphology

Answer 15

• usually small, solitary, encapsulated

- cut surface is usually bright yellow, reflecting high lipid content

Question 16

what is the clinical course of hyperaldosteronism?

Answer 16

• hypertension
• hypokalemia
• high aldosterone, low blood renin

Question 17

what are the different types of adrenocortical insufficiency?

Answer 17

• primary acute adrenocortical insufficiency (adrenal crisis)
• primary chronic adrenocortical insufficiency (addison)
• secondary adrenocortical insufficiency

Question 18

what is waterhouse-friedrichsen syndrome? what is the result? what is the typical patient population?

Answer 18

overwhelming septicemic infection due to neisseria meningitidis infection

results in massive adrenal hemorrhage with adrenal insufficiency

children

Question 19

what is the morphology of addison disease?

Answer 19

autoimmune adrenalitis producing

• small glands
• lipid depletion of cortex
• variable lymphocytic infiltrate in cortex

Question 20

addison disease: signs and symptoms

Answer 20

fatigue 
anorexia 
nausea 
cutaneous hyperpigmentation 
hypotension 
elevated ACTH 
high potassium 
low sodium

Question 21

secondary adrenocortical insufficiency: typical morphology

Answer 21

• variable degrees of atrophy of the adrenal cortex

- sparing of the zona glomerulosa and medulla

Question 22

what are the aldosterone and pigmentation levels like in secondary adrenocortical insufficiency?

Answer 22

NO hyperpigmentation 
normal aldosterone (it is independent of ACTH)

Question 23

which neuroendocrine markers are positive in pheochromocytoma?

Answer 23

synaptophysin

chromogranin

Question 24

pheochromocytoma: histology

Answer 24

mature polygonal to spindle-shaped medullary-type cells containing basophilic cytoplasmic granules arrayed in trabeculae or small nests

Question 25

where do neuroblastomas arise?

Answer 25

adrenal medulla or in extra-adrenal paraganglia

Question 26

neuroblastomas involve an amplification of what oncogene?

Answer 26

N-MYC

Question 27

neuroblastoma: histology

Answer 27

• “small blue cell tumors”

- homer-wright rosettes (neuroblasts located around a central space)

Question 28

neuroblastoma: clinical findings

Answer 28

• palpable abdominal mass
• diastolic hypertension
• commonly metastasizes to skin and bones
• increased vanillylmandelic acid (VMA)
• increased metanephrines
• increased homovanillic acid (HVA)

Question 29

neuroblastoma: presentation

Answer 29

“blueberry muffin baby”