Adrenal Pathology Singh Flashcards

1
Q

What are the three layers to the adrenal glands and what is secreted?

A
  • Capsule
  • Zona Glomerulosa → Aldosterone
  • Zona Fasciculata → Cortisol
  • Zona Reticularis → Androgens
  • Medulla
  • GFR → salty, sweet, sex
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2
Q

What happens to the adrenal glands with ACTH dependent cushing syndrome?

A

Adrenal reactive hyperplasia

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3
Q
A
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4
Q

Primary Hyperaldosteronism (Conn’s syndrome) symptoms

A
  • Htn
  • Hypokalemia
  • Hypomagnesemia
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5
Q

Most common cause of hyperaldosteronism?

A

Idiopathic

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6
Q

SEcondary hyperaldosteronism?

A
  • Diuretics
  • decreased renal perfusion
  • arterial hypovolemia
  • pregnancy
  • renin secreting tumors
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7
Q

What is congenital adrenal hyperplasia?

A
  • Inherited error of metabolism (auto recessive)
  • Impaired feedback to hypothalamus/pituitary with resultant hyperplasia
  • 90-95% of cases caused by 21-hydroxylase deficiency
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8
Q

What is the most significant form of CAH?

A
  • Salt wasting syndrome when you have a complete lack of the enzyme so there are no mineralocorticoids or cortisol
  • can diagnose in females at birth due to viriliztion
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9
Q

What happens with a partial lack of enzyme 21 hydroxylase

A
  • some mineralocorticoids and cortisol but not enough to prevent over production of ACTH
  • Virilization can occur
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10
Q

Nonclassiv late onset adrenal virilism?

A
  • most common
  • partial lack of 21-hydroxylase
  • leading to percocious puberty and excess acne and hirsutism at time of puberty
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11
Q

How do we treat 21-hydroxylase deficiency?

A
  • Glucocorticoid administration → provides the negative feedback for ACTH suppression
  • mineralocorticoids as needed
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12
Q

Adrenocortical insufficiency primary and secondary cauess?

A

Primary:

  • loss of cortical cells
  • defect in hormonoogenesis

Secondary:

  • hypothalamic pituitary dissease
  • HPA suppression by extra adrenal steroid source
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13
Q

What happens to the adrenal glands when you are on steroids?

A
  • on corticosteroid therapy the adrenals are atrophied, that is why you need to taper steroids to give them a chance to “wake up”
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14
Q

What can cause adrenal hemorrhage?

A
  • Sepsis → waterhouse friderichsen syndrome
  • neonatal period
  • trauma
  • post surgical patients
  • coagulopathy
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15
Q

What are the sx of acute adrenal insufficiency

A
  • hypotension → refractory to volume repletion
  • abdominal pain
  • fever
  • n/v
  • hyperkalemia
  • hyponatremia
  • hypoglycemia
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16
Q

Primary chronic adrenocortical insufficiency?

A
  • Long duration of malaise fatigue anorexia and weight loss
  • joint pain
  • hyperpigmentation of the skin
17
Q

Primary adrenocortical insufficiency other name and causes?

A
  • Addison’s disease
  • 1800’s used to be caused by TB
  • Most common cause in developed countries and world wide is autoimmune
18
Q

Autoimmune Polyendocrine syndrome type 1?

A
  • AIRE gene mutation
  • adfrenalitis
  • parathyroiditis
  • hypogonadism
  • pernicioius anemia
  • mucocutaneous candidiasis
  • ectodermal dystrophy

APECED

19
Q

What is autoimmune polyendocrine syndrome type 2?

A
  • adrenalitis
  • thyroiditis
  • DM type 1
20
Q

Compare size in adrenal cortical adenoma and carcinoma?

A

Carcinomas are larger than adenoma and weigh more than 200 grams

21
Q

Presentation of adrenal cortical adenoma?

A

Incidental finding on radiograph, they’re usually functional

22
Q

Presentation of adrenal cortical carcinomas?

A

Indicental finding on radiograph, functional, and compresses/invades adjacent structures. Also causes virilization

23
Q

what is this

A

Left is adenoma and right is adrenal carcinoma with large cells and mitotic figures

24
Q

What cell makes up the adrenal medulla and its function?

A

Chromaffin cells from the neural crest which are responsible for catecholamine secretion

25
Describe pheochromocytoma rule of 10's
* presents with profound htn from catecholamine secretion from the tumor * 10% rule: * 10% are extra adrenal (paraganglioma) * 10% are b/l * 10% in kids * 10% malignant (only can tell from mets) * 10% NOT assoc. with htn * 25% rule: 25% are familial
26
What is the classic triad with pheochromocytoma?
* Headache * Palpitation * Diaphoresis
27
Complications of pheochromocytoma?
* acute related to catecholamine surge * chronic related to cardiomyopathy
28
What is the histology for pheochromocytoma?
Zellballen
29
How do you diagnose pheochromocytoma?
urine and plasma metanephrines
30
What is a myelolipoma?
Benign bone marrow and fat tumor that can vary in size and present with hemorrhage
31
What are adrenal incidentalomas?
* Incidentally discovered adrenal nodule- need to determine what it is * need to assess the size \>4cm * blood tests: * cortisol * aldosterone * metanephrines * CT enhancement characteristics
32
What tumros occur with Multiple endocrine neoplasia 1? What is the germline mutation?
* Menin is the mutation * Primary hyperparathyroidism * Pancreatic endocrine tumors * Pituitary adenomas * lactotroph and somatotroph *3 P's*
33
MEN 2A tumors and mutationo?
* RET (gain of function) * Pheochromocytoma * Medullary thiyroid carcinoma * Parathyroid hyperplasia
34
MEN 2B tumors and mutation?
* RET (gain of function\_) * Medullary thyroid carcinoma * Pheochromocytoma * Mucosal neuroma
35
What mutation makes up familial medullary thyroid carcinoma?
RET gain of function, but all you get is the thyroid carcinoma
36
When do thyroids need to be removed if you have MEN2
By age 10 at the latest to prevent thyroid carcinoma
37
Prognosis of MEN syndrome tumors?
Aggressive and recurrent
38
What tumors can the pineal gland get and what does it secret?
* Secretes melatonin Tumors include: (very rare) * Germ cell tumors * Pineocytoma * Pineoblastoma