Lecture 20 - Pathology of the Adrenal Gland

Question 1

The overwhelming cause for Cushing Syndrome is from _______ (endogenous or exogenous?) glucocorticoids.

Answer 1

Exogenous

Question 2

_____-secreting pituitary _______ are the most common cause of Endogenous hypercortisolism. This is more common in _____ (sex) and young adults.

Answer 2

ACTH-secreting pituitary Adenomas

Women

Question 3

The most common cause of ectopic ACTH secretion leading to Cushing syndrome is paraneoplastic syndrome. Specifically, which cancer type is the most common cause? Keep in mind this is more common in ____ (sex?) in their 40s-50s.

Answer 3

Small cell lung cancer

Men

Question 4

While Adrenocortical Hyperplasia results in hypercortisolism INDEPENDENT of ACTH, it is not autonomous. What does it depend on?

Answer 4

FSH and LH

Question 5

Micronodular hyperplasia is distinct from Macronodular both in size of the nodules and in their color. How do they appear?

Answer 5

Micronodular –> small, BROWN colored nodules with cortical atrophy.

Question 6

Diagnosis of Cushing syndrome requires increased 24 hour free Cortisol and loss of normal _____ pattern of cortisol secretion.

Answer 6

Diurnal

Question 7

____ ____ hyperaldosteronism is the most common cause of Primary hyperaldosteronism. _____ syndrome is the term to describe primary hyperaldosteronism caused by a solitary neoplasm.

Glucocorticoid-remediable hyperaldosteronism is an uncommon cause of Primary hyperaldosteronism –> in some families it stems from a rearrangement of CYP11B2 with CYP11B1 (responsive to ACTH) on Chromosome ____.

Answer 7

Bilateral Idiopathic

Conn

8

Question 8

Aldosterone producing Adenomas are almost always _____ (solitary or multifocal?). They’re most common in _____ (sex?) in the 3rd to 4th decades of life. On histology, look for pink cellular inclusions indicative of ______ treatment.

Answer 8

Solitary

Women

Spironolactone

Question 9

DHEA and Androstenedione are androgens produced in the adrenal cortex zona reticularis and fasciculata –> they can be converted in the periphery to ______.

Keep in mind adrenocortical neoplasms associated with virilization (excess androgens) are more often _______ (adenomas or carcinomas?)

Answer 9

Testosterone

Carcinomas

Question 10

________ deficiency is the most common cause of congential adrenal hyperplasia. It results from a mutation in ______.

Answer 10

21-hydoxylase

CYP21A2

Question 11

Waterhouse-Friderichsen syndrome is a rare cause of Primary Adrenal Insufficiency that results from massive adrenal _______ secondary to severe sepsis (usually N. meningitidis).

Answer 11

Hemorrhage

Question 12

Autoimmune Adrenalitis is the most common cause of Primary Adrenal Insufficiency (AKA _______ disease). It can occur in one of two clinical settings.

1. APS1 (Autoimmune polyendocrine syndrome type 1) caused by mutations in _____ gene on chromosome 21q22. Look for patients to present with many symptoms of autoimmunity (i.e. candidiasis).
2. APS2 which is characterized by adrenal insufficiency, autoimmune ______, and type ___ diabetes.

Answer 12

Addison’s

AIRE gene

Autoimmune thyroiditis

Type 1 diabetes

Question 13

Autoimmune adrenal insufficiency will show ____ infiltrate in the cortex with an intact medulla.

Answer 13

Lymphocytic

Question 14

The hallmark characteristic of infectious Adrenal insufficiency is presence of necrotizing ______ (think TB).

Answer 14

Granulomas

Question 15

Are adrenal carcinomas or adenomas more likely to be large and functional?

Answer 15

Carcinomas

Question 16

Are adrenal carcinomas more likely to be a result of primary or metastatic disease?

Answer 16

Mets!!

Question 17

One of the hallmarks of Pheochromocytoma is the feeling of _____ _____. Also look for HTN in upward of 90% of cases (should make sense since Epi and Norepi are produced by chromaffin cells).

Answer 17

Impending doom

Question 18

How is the malignant potential of Pheochromocytoma determined?

Answer 18

It can’t be determined Histologically! You actually have to wait for it to metastasize…

Question 19

________ is the most common diagnosed tumor in infancy. Mutations in _____ are a major cause of familial predisposition for these tumors.

Answer 19

Neuroblastoma

ALK (Anaplastic Lymphoma Kinase)

Question 20

Neuroblastomas can become very large, with 40% of them occurring within the adrenal ______. Look for small round _____ (color?) cells on histology with occasional Homer-Wright _______ (cells surrounding pink staining neuropil).

Answer 20

Medulla

Blue

Pseudo-rosettes

Question 21

One of the worst prognostic factors for Neuroblastoma is amplification of _____, an oncogene –> High Trk__ expression is associated with LOW amplification, while High Trk___ is associated with HIGH amplification.

Answer 21

MYCN

TrkA

TrkB