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2021-2022 Endo Review Rearr. > 179b/180b/183b - Adrenal Pathology, Adrenal Medulla, Adrenal Cortex > Flashcards

179b/180b/183b - Adrenal Pathology, Adrenal Medulla, Adrenal Cortex Flashcards

1
Q

What 3 things regulate aldosterone secretion?

A
  • RAAS
  • ​Hyperkalemia
  • ACTH

ACTH stimulates desmolase, the 1st step in aldosterone synth; however, not a primary regulater of aldosterone synthesis

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2
Q

Where do parasympathetic paragangliomas usually arise?

A

Parasympathetic nerves in the head, neck, and upper mediastinum

“Head and Neck paragangliomas”

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3
Q

Which pharmacologic agent can be used to block glucocorticoid receptors?

A

Mifepristone

(progesterone receptor/glucocorticoid receptor antagonist)

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4
Q

11-beta-hydroxylase is required to synthesize aldosterone

Why don’t people with 11-beta-hydroxylase deficiency have low blood pressure?

A

Deoxycorticosterone can activate aldosterone receptors, leading to water and Na+ reabsorption -> hypertension

Deoxycorticosterone is the substrate of 11-beta-hydroxylase in aldosterone synthesis

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5
Q

Which enzyme converts norepinephrine to Epinephrine?

Where is this enzyme found?

A

Phenylethanolamine N-methyl transferase

ONLY in the adrenal medulla

=> no other organ can secrete epinephrine

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6
Q

Which tumors most commonly metastasize to the adrenal gland? (2)

A

Renal cancer

Lung cancer

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7
Q

Which genetic syndromes are associated with pheochromocytoma? (3)

A
  • MEN 2
  • Von Hippel-Lindau
  • NFM-1
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8
Q
A

A. Zona Fasiculata

  • Mifepristone is also a glucocorticoid receptor antagonist
  • Cortisol is produced in the zona fasiculata
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9
Q

What tumors are associated with Von Hippel Lindau? (6)

A
  • CNS Hemangioblastoma
  • Pancreatic cyts
  • Pheochromocytomas
  • Retinal angioma
  • Renal cysts
  • Renal cell carcinomas

The Hippo might need CPPRRR

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10
Q

What tumors are common to MEN-2A and MEN-2B?

How can they be differentiated?

A
  • Both = medullary tumors
    • Medullary thyroid carcinoma
    • Pheochromocytoma
    • Caused by activating mutation in the RET proto-oncogene
  • MEN-2A
    • Parathyroid hyperplasia
    • Less severe than MEN-2B
  • MEN-2B
    • Marfanoid appearance (Thin face, big lips, tongue nodules)
    • Mucosal neuroma
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11
Q

What is the effect on insulin and glucagon secretion of:

  • alpha-adrenergic receptor activation:
  • beta-adrenergic receptor activation:
A
  • alpha-adrenergic receptor activation: Inhibits secretion
  • beta-adrenergic receptor activation: Stimulates secretion
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12
Q

What is the most common adrenal tumor?

A

Adrenal cortial adenoma

(Benign)

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13
Q

Describe the gross and histological findings of an adrenal cortical adenoma

A

Like the normal adrenal gland

  • Gross: Yellow-ish
  • Histological No necrosis, hemorrhage, or mitosis
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14
Q

What cells are ganglioneuromas derived from?

A

Neural crest cells

  • Ganglion cells
  • Schwannian stroma
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15
Q

What is the most common caue of isolated hypoaldosteronism?

A

Hyporeninemic hypoaldosteronism

(Type IV renal tubular acidosis)

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16
Q

What is the name for a catecholamine-producing tumro in the…

Adrenal medulla?

Outside of the adrenal medulla?

A
  • Adrenal medulla: pheochromocytoma
    • Produces epinephrine
  • Outside of the adrenal medulla: paraganglioma
    • ​Produces norepinephrine
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17
Q

What is the embryonic origin of the adrenal medulla?

A

Neural crest cells

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18
Q

Describe the characteristics of NFM-1 (5)

A
  • Neurofibroma
  • Café-au-lait spots
  • Iris harmatoma
  • Axillary and inguinal freckling
    • Pheochromocytoma in ~2%
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19
Q

What genetic mutation is associated with MEN-2?

How is it inherited?

A

Germline point mutation in the RET proto-oncogene

Autosomal dominant

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20
Q

Are sympathetic or parasypathetic paragangliomas more likely to be symptomatic?

A

Sympathetic

21
Q

Describe the pathological findings of an adrenal cortical carcinoma

A
  • Hemorrhage and necrosis
  • Larger; >6.5cm, >100gm
  • Invades surrounding tissue

Basically, if it looks abnormal its a carcinoma

22
Q

What kind of cells are found in the paraganglia of the adrenal gland

A

Neuroendocrine cells that stain with chromaffin staining

(Similar to adrenal medulla cells)

23
Q

What genetic mutation ins associated with Von Hippel-Lindau?

How is it inherited?

A

Mutated vHL gene (tumor suppressor)

Autosomal dominant

24
Q

What are the layers of the adrenal cortex?

What do they secrete?

A
  • Zona Glomerulosa secretes mineralocoricoids (aldosterone)
  • Zona Fasciculata secretes glucocorticoids (cortisol)
  • Zona Reticularis secretes androgens (testosterone)
25
Q

What are the 2 main types of medullary tumors?

A
  • Pheochromocytoma
    • ​Tumor in the adrenal medulla
  • Paraganglioma
    • ​Tumor in the extra adrenal sympathetic and parasympathetic ganglia

Both tumors arise from chromaffin cells

26
Q

What cells are neuroblastomas derived from?

A

Neural crest cells

(Same as ganglioneuroma, but neuroblastoma has characteristic round blue cells and occurs in children)

27
Q

Is cortical hyperplasia typically unilateral or bilateral?

A

Bilateral

28
Q

Which pharmacologic agents can be used to block mineralcorticoid receptors? (2)

A

Spironolactone

Eplerenone

29
Q

How do we measure catecholamine levels?

A

Look for metabolites (metanephrines) in the urine

Cannot directly measure serum levels

30
Q

What cells are pheochromocytomas derived from?

A

Chromaffin cells of the adrenal medulla

31
Q

How does black licorice affect blood pressure

A

Licorice will increase blood pressure

  • Contains glycyrrhizinic acid, which blocks 11-beta-hydroxysteroid dehydrogenase II
  • Normally, 11-beta-hydroxysteroid dehydrogenase inactivates cortisol to cortisone
  • Inhibition -> more coritosl -> activates aldosterone receptors
  • -> Hypertension
32
Q

What is the defining gross feature of diffuse cortical hyperplasia?

A

Diffuse, symmetric enlargement of the cortex

(Ex: Congenital adrenal hyperplasia)

33
Q

What does the adrenal medulla produce?

A

Catecholamines

  • Inside of the medulla = epinephrine
    • ​Contains the enzyme needed to convert NE to Epi
  • Extra-adrenal tissue = norepinephrine
34
Q

Which image shows adrenal cortical carcinoma?

Which shows adenoma?

A
35
Q

What are “zellballen”?

What adrenal tumor are they associated with?

A

Nests of large tumor cells

Pheochromocytoma

36
Q

Are neuroblastomas benign or malignant?

A

Malignant

(Small round blue cells)

37
Q

Which medullary tumor carries the highest risk of malignancy?

A

Paraganglioma

38
Q

A patinet is scheduled to have a pheochromocytoma removed in 2 weeks.

What should you give them before the surgery?

A
  • FIRST give alpha blocker (phenoxybenzamine) for 1 week
    • Lower BP
    • Expand blood volume - Increase Na+, fluid intake
  • THEN give beta blocker (propanolol)

Goal is to prevent hemodynamic collapse when the tumor is removed

39
Q

What are the classic histological findings of neuroblastoma? (2)

A

Small round blue cells

Homer-Wright Rosettes

40
Q

Which is the active form: cortisol or cortisone?

Which enzyme converts one to the other?

A

Cortisol is active

11-beta-hydroxysteroid dehydrogenase

  • Type I: activates cortisone to cortisol
    • Liver, lung, omental fat
  • Type II: inactivates cortisol to cortisone
41
Q

Where do sympathetic gangliomas usually arise?

A
  • Chromaffin cells of the paraganglia
  • Sympathetic chains of the chest
42
Q

What is the effect of Epinephrine/NE on blood glucose?

A

Epinephrine and NE -> increased blood glucose

Stimulates gluconeogenesis, glycogenolysis

43
Q

Which population is associated with neuroblastoma?

A

Children

44
Q

Are ganglioneuromas benign or malignant?

A

Benign

(Also, not hormonally active)

45
Q

Which pharmacologic agents can be used to block steroidogenesis? (2)

A

Ketoconazole (blocks demolase, 17,20 lyase, 17-alpha-hydroxylase

Metyrapone (blocks 11-beta-hydroxylase)

46
Q

What clinical signs/symptoms (altogether) would increase your suspicion for pheochromocytoma or paraganglioma? (5)

A
  • Headaches
  • Palpitations
  • Diaphoresis
  • HTN
  • Abdominal pain
47
Q

Which hormone will be elevated in a patinet with an extra-adrenal pheochromocytoma?

A

Norepinephrine

Extra-renal pheo = paraganglioma

48
Q

Which mutation is associated with paraganglioma genetic syndromes?

What is its normal function?

A

Succinate Dehydrogenase (SDH)

  • Component of the mitochondrial electron transport chain complex 2
  • Catalyzes the oxidation of succinate -> fumarate in the Krebs cycle
49
Q

How does cortisol impact red blood cell production?

A

Cortisol is necessary for RBC production

(but also suppresses all aspects of the immune system)

2021-2022 Endo Review Rearr. (19 decks)

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