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Endo by Minnie: AH Version > 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
What are the 2 main types of medullary tumors?
* **Pheochromocytoma** * **​**Tumor **in the adrenal medulla** * **Paraganglioma** * **​**Tumor in the **extra adrenal sympathetic and parasympathetic ganglia** Both tumors arise from **chromaffin cells**
26
What cells are neuroblastomas derived from?
Neural crest cells (Same as ganglioneuroma, but neuroblastoma has characteristic round blue cells and occurs in children)
27
Is cortical hyperplasia typically unilateral or bilateral?
Bilateral
28
Which pharmacologic agents can be used to block mineralcorticoid receptors? (2)
Spironolactone Eplerenone
29
How do we measure catecholamine levels?
Look for metabolites (metanephrines) in the urine ## Footnote *Cannot directly measure serum levels*
30
What cells are pheochromocytomas derived from?
Chromaffin cells of the adrenal medulla
31
How does black licorice affect blood pressure
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
What is the defining gross feature of diffuse cortical hyperplasia?
Diffuse, symmetric enlargement of the cortex (Ex: Congenital adrenal hyperplasia)
33
What does the adrenal medulla produce?
Catecholamines * Inside of the medulla = **epinephrine** * **​**Contains the enzyme needed to convert NE to Epi * Extra-adrenal tissue = **norepinephrine**
34
Which image shows adrenal cortical carcinoma? Which shows adenoma?
35
What are "zellballen"? What adrenal tumor are they associated with?
Nests of large tumor cells Pheochromocytoma
36
Are neuroblastomas benign or malignant?
Malignant (Small round blue cells)
37
Which medullary tumor carries the highest risk of malignancy?
Paraganglioma
38
A patinet is scheduled to have a pheochromocytoma removed in 2 weeks. What should you give them before the surgery?
* 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
What are the classic histological findings of neuroblastoma? (2)
Small round blue cells Homer-Wright Rosettes
40
Which is the active form: cortisol or cortisone? Which enzyme converts one to the other?
**Cortisol is active** 11-beta-hydroxysteroid dehydrogenase * Type I: **activates cortisone to cortisol** * Liver, lung, omental fat * Type II: **inactivates cortisol to cortisone**
41
Where do sympathetic gangliomas usually arise?
* Chromaffin cells of the paraganglia * Sympathetic chains of the chest
42
What is the effect of Epinephrine/NE on blood glucose?
Epinephrine and NE -\> **increased blood glucose** ## Footnote *Stimulates gluconeogenesis, glycogenolysis*
43
Which population is associated with neuroblastoma?
Children
44
Are ganglioneuromas benign or malignant?
Benign | (Also, not hormonally active)
45
Which pharmacologic agents can be used to block steroidogenesis? (2)
**Ketoconazole** (blocks demolase, 17,20 lyase, 17-alpha-hydroxylase **Metyrapone** (blocks 11-beta-hydroxylase)
46
What clinical signs/symptoms (altogether) would increase your suspicion for pheochromocytoma or paraganglioma? (5)
* Headaches * Palpitations * Diaphoresis * HTN * Abdominal pain
47
Which hormone will be elevated in a patinet with an extra-adrenal pheochromocytoma?
**Norepinephrine** Extra-renal pheo = paraganglioma
48
Which mutation is associated with paraganglioma genetic syndromes? What is its normal function?
Succinate Dehydrogenase (SDH) * Component of the mitochondrial electron transport chain complex 2 * Catalyzes the oxidation of **succinate -\> fumarate** in the Krebs cycle
49
How does cortisol impact red blood cell production?
Cortisol is necessary for RBC production (but also suppresses all aspects of the immune system)

Endo by Minnie: AH Version (19 decks)

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