Adrenal Pathology

Question 1

What is shape of right adrenal? What is shape of left adrenal?

Answer 1

Right is pyramidal in shape. Left is more crescent in shape

Question 2

What do layers of cortex look like?

Answer 2

Glomerulosa is more purple
Fasciculata is largest layer; most clear (lipids for steroid synthesis)
Reticularis is pinker

Question 3

What is pathology of Addison’s disease (5)?

Answer 3

Destruction of cortical cells
Chronic inflammatory cells (if autoimmune)
Infectious agents (i.e granulomatous inflammation)
Metastatic tumors
Amyloid deposition

Question 4

What are clinical features of Addison’s disease? (8)

Answer 4

Fatigue, malaise, weakness, anorexia, vomiting, weight loss, hyper pigmentation, postural hypotension

Question 5

What are causes of Addison’s disease (5 categories)

Answer 5

Congenital: Agenesis/dysgenesis
Autoimmune
Infections: TB, Strep Pneumo
Metastases to adrenals
Amyloid deposition

Question 6

Causes of Cushing’s syndrome: (3)

Answer 6

Bilateral hyperplasia: Pituitary ACTH secreting adenoma, ectopic ACTH
Adrenal cortical adenoma
Adrenal cortical carcinoma

Question 7

Which tumor is most often responsible for ectopic ACTH?

Answer 7

Small cell lung carcinoma

Question 8

What is most common cause of Cushing’s syndrome? Is it different for adults vs. children?

Answer 8

Adults: Hyperplasia
Children: Carcinoma

Question 9

What is breakdown of appearance of adrenal cortical hyperplasia?
More common in men or women? Weight?

Answer 9

Simple diffuse: 65%, women, 6-12g
Bilateral nodular: 20%, women, 10-15
Ectopic hyperplasia: 15%, males, 20g

Question 10

What is gross pathology of adrenal cortical adenoma? (3)

Answer 10

Unilateral and solitary
Well circumscribed
Yellow tan surface

Question 11

Describe the microscopic pathology of Cushing’s Syndrome (3)

Answer 11

Mixture of growth patterns: nests, cords, solid
Cells have clear cytoplasm– resemble zona fasciculate
No mitoses or atypical cells

Question 12

Describe the gross pathology of Adrenal cortical carcinoma (3)

Answer 12

Large bulky tumor
Hemorrhage and necrosis
Invasion into adjacent structures

Question 13

Describe microscopic pathology of adrenal cortical carcinoma (6)

Answer 13

Invasion of nearby organs
Necrosis
Pleomorphism
Capsule invasion
Vascular invasion
Metastases

Question 14

What are the causes of hyperaldosteronism? (2)

Answer 14

Adenoma: 65%

Bilateral hyperplasia: 35%

Question 15

Describe pathology of aldosterone excess causing adrenal cortical adenoma

Answer 15

Gross: unilateral, solitary, golden-yellow cut surface
Microscopic: Multiple cell types: mostly ZF cells but some have ZG cells

Question 16

What are clinical features of aldosterone excess? (4)

Answer 16

Hypertension
Muscle weakness, fatigue, polyuria
Periodic paralysis
Cardiac arrhythmias

Question 17

What are causes of androgen excess?

Answer 17

Congenital adrenal hyperplasia

Adrenal cortical adenoma/carcinoma (rare)

Question 18

What are clinical features of Adrogenital syndrome? Describe for men and women

Answer 18

Women: male hair pattern with facial hair
Men: gynecomastia, decreased libido, feminized hair pattern, testicular atrophy

Question 19

What is pathophysiology of congenital adrenal hyperplasia?

Answer 19

Deficiency of enzymes required for biosynthesis of MCs/GCs–>lots of ACTH from pituitary–>increased androgens

Question 20

What are causes of adrenal medulla hyper function? (3)

Answer 20

Pheochromycytoma
Hyperplasia
Neuroblastoma

Question 21

Describe epidemiology for pheochromocytoma (2): Incidence and demographics

Answer 21

2-8M per year

No sex/age predilection–rare in children

Question 22

What are clinical features of pheochromocytoma (big list)?

Answer 22

Excess catecholamines: hypertension, palpitations, headaches, palpitations, headaches, diaphoresis, flushing, anxiety, nausea, constipation, pain, epistaxis

Question 23

How much of pheochromocytomas are familial? What is inheritance?

Answer 23

Between 20-35% pheochromocytomas are familial.

Mutations are passed in an autosomal dominant fashion

Question 24

What are familial syndromes associated with pheochromocytoma? (3) Name some germline mutations: (5)

Answer 24

Multiple endocrine neoplasia, Von Hippel Lindau syndrome, neurofibromatosis

Mutations: SDHB, SDHD, VHL, RET, NF1

Question 25

Describe difference between sporadic and familial pheochromocytomas on pathology

Answer 25

Sporadic: unilateral
Familial: bilateral or multiple tumors

Question 26

Describe gross findings of pheochromocytoma (3)

Answer 26

Big
Well circumscribed
Vascular

Question 27

Describe microscopic findings in pheochromocytoma (4)

Answer 27

Growth pattern: nested, solid
Organoid pattern: round group of cells supported by vascular network
“Salt and pepper” nuclei: neuroendocrine chromatin
Malignancy: necrosis, mitoses, vascular invasion

Question 28

What are the multiple endocrine neoplasia syndromes?

Answer 28

MEN1 (Wermer syndrome)
MEN2A (Sipple syndrome)
MEN2B

Question 29

Name location of neoplasia in MEN1 (6)

Answer 29

Main ones: Pituitary, parathyroid, pancreatic islets

Others: adrenal cortex, lung, thymus

Question 30

How is clinical prognosis for MEN1 determined?

Answer 30

Related to pancreatic lesions, which can be malignant

Question 31

What mutations are involved in MEN2A/2B? What is most important prognostic lesion?

Answer 31

Mutation in ret proto-oncogene

Medullary thyroid carcinoma– can be fatal

Question 32

In MEN2A, observe neoplasia/hyperplasia of ______ (3)

Answer 32

Thyroid C cells
Adrenal medulla
Parathyroids (15-25%)

Question 33

In MEN2B, observe neoplasia/hyperplasia of ______ (4)

Answer 33

Thyroid C cells
Adrenal medulla
Neural tissue of oral/GI systems
Skeletal and eye lens abnormalities