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ENRP Exam II > Adrenal Pathology Singh > Flashcards

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
Q

Describe pheochromocytoma rule of 10’s

A
  • 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
Q

What is the classic triad with pheochromocytoma?

A
  • Headache
  • Palpitation
  • Diaphoresis
27
Q

Complications of pheochromocytoma?

A
  • acute related to catecholamine surge
  • chronic related to cardiomyopathy
28
Q

What is the histology for pheochromocytoma?

A

Zellballen

29
Q

How do you diagnose pheochromocytoma?

A

urine and plasma metanephrines

30
Q

What is a myelolipoma?

A

Benign bone marrow and fat tumor that can vary in size and present with hemorrhage

31
Q

What are adrenal incidentalomas?

A
  • 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
Q

What tumros occur with Multiple endocrine neoplasia 1? What is the germline mutation?

A
  • Menin is the mutation
  • Primary hyperparathyroidism
  • Pancreatic endocrine tumors
  • Pituitary adenomas
    • lactotroph and somatotroph

3 P’s

33
Q

MEN 2A tumors and mutationo?

A
  • RET (gain of function)
  • Pheochromocytoma
  • Medullary thiyroid carcinoma
  • Parathyroid hyperplasia
34
Q

MEN 2B tumors and mutation?

A
  • RET (gain of function_)
  • Medullary thyroid carcinoma
  • Pheochromocytoma
  • Mucosal neuroma
35
Q

What mutation makes up familial medullary thyroid carcinoma?

A

RET gain of function, but all you get is the thyroid carcinoma

36
Q

When do thyroids need to be removed if you have MEN2

A

By age 10 at the latest to prevent thyroid carcinoma

37
Q

Prognosis of MEN syndrome tumors?

A

Aggressive and recurrent

38
Q

What tumors can the pineal gland get and what does it secret?

A
  • Secretes melatonin

Tumors include: (very rare)

  • Germ cell tumors
  • Pineocytoma
  • Pineoblastoma

ENRP Exam II (9 decks)

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