Adrenal Gland - Guerin Flashcards by Alex Hamidi

CFs of Addison’s?

Labs?

Begins insidiously
Progressive weakness and fatigue
GI disturbance
Hyperpigmentation

Hyponatremia, hyperkalemia

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Major morphology of pheochromocytomas?

Avg weigh 100gm, range from 1-4000

Small to large hemorrhagic masses

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What are the sex steroids found in the adrenal gland cortex?

Made where?

Estrogen and androgen

Zona reticularis

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CFs of pheochromocytomas?

Dx?

Tx?

HTN, palpitations, sweating

Elevated urine VMA and metanephrines

Surgery after admin adrenergic-blocking agent

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What is the most common site of gastrinomas in MEN1?

What other tumors?

Duodenum

Carcinoid, thyroid, adrenocortical, lipomas

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A virilizing neoplasm is more likely a what?

Hyperaldosteronism and Cushing?

Carcinoma

Functional adenoma

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Endogenous hypercortisolism w/ACTH-depended Cushing syndrome causes what?

Diffuse hyperplasia

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Stress can precipitate what in Addison’s?

Acute adrenal crisis

Hypoglycemia can also occur

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Familial medullary thyroid cancer is a variant of what?

Pts are what?

Clinical course?

MEN2A

Older

Indolent

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What structures are found in the adrenal medulla?

Chromaffin cells
Medullary veins
Splanchnic nerves

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MEN2B has what tumors?

What mutation?

Medullary carcinoma of the thyroid (MORE aggressive than 2A), pheochromocytomas, neuromas, Marfanoid habitus

RET
Sporadic medullary carcinoma is aggressive and has adverse prognosis

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What are 2 familial cancer syndromes w/inc risk of adrenocortical carcinomas?

Li-Fraumeni syndrome (TP53 mutation)

Beckwith Wiedemann

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Phechromocytomas are composed of what?

Rule of 10s?

Chromaffin cells

10% have:
paragangliomas
Sporadic are B/l
Malignant
No HTN

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Glucocorticoids induce ______ and inhibit _____?

Gluconeogenesis

Uptake of glucose by cells

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90% of CAH occurs due to what?

Mutation where?

21-Hydroxy deficiency

CYP21A2

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Adenomas causing hyperaldosteronism are usually solitary or multiple? What location?

Size?

Color?

Characteristic feature?

Solitary, Left > right

Small (> 2cm)

Bright yellow grossly

Spironolactone bodies (eosinophilic cytoplasmic inclusions)

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Any disorder of the hypothalamus and pituitary that reduces the output of ACTH can cause what?

What else?

Secondary Adrenocortical Insufficiency

Exogenous glucocorticoids

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What causes secondary Adrenocortical insufficiency?

Def of ACTH

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What is the most common cause of an adrenocortical neoplasm?

Called what?

What demographic?

Adenoma

Conn syndrome

Middle aged women (30-40 y/o)

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Primary adrenocortical neoplasms occur most often in whom?

Functioning tumors cause what?

Women aged 30-50

Atrophic adrenal cortex and contralateral adrenal gland

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What system is composed of adrenal medulla?

Paraganglion

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The pineal gland secretes what?

Although rare, what tumors are there?
Resemble what?

Melatonin

Germ cell tumors
Testicular seminomas

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tumors of MEN syndromes are typically what compared to sporadic tumors?

What stage precedes tumor?

More aggressive

Asymptomatic Hyperplasia

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Aldosterone released in response to the activation of RAAS by Inc renin is what?

Secondary hyperaldosteronism

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Inability to convert progesterone into deoxycorticosterone is what? In females presents when? Males? CFs?

Salt-wasting syndrome At birth/in utero Males 5-15 days after birth Hyponatremia and hyperkalemia -> acidosis, HTN, CV collapse

Primary acute adrenocortical insufficiency occurs in what settings?

Crisis due to stress Exogenous corticosteroids w/rapid withdrawal or failure to INC dose w/stress Massive adrenal hemorrhage due to damage of adrenal cortex

What happens when the pituitary shows Crooks hyaline change? Found in what?

Basophilic cytoplasm becomes homogeneous and pale Cushing's

What is the most common CF in hyperaldosteronism? Effects on CV system? What else?

HTN LVH -> Dec diastolic volume, stroke and MI Weakness, parasthesias, visual disturbances

Tx of CAH?

Exogenous glucocorticoids and mineralcorticoids for salt-wasting variants

Describe adrenocortical adenomas

Well-circumscribed nodular Yellow to yellow-brown Cortex adjacent to nonfx adenoma is normal

Describe morphology of CAH

Adrenals are b/l hyperplastic | Cortex thickened and nodular, and brown

Adrenocortical carcinomas invade what? Pt survival? Size?

Adrenal vein, vena cava, lymphatics 2 yrs Large and invasive, > 20 cm

What has the morphology of irregular shrunken glands w/a cortex containing scattered residual cortical cells in a collapsed network of ct?

Primary autoimmune adrenalitis

What is the most common reason for ectopic ACTH-dependent Cushing Syndrome?

Small-cell carcinoma of the lung

What 3 things can cause primary hyperaldosteronism?

Adrenocortical neoplasm Bilateral idiopathic hyperaldosteronism Glucocorticoid-remediable hyperaldosteronism

pineal gland composed of what? What functions?

Pineocytes Photosensory and neuroendocrine

Tx of bilateral hyperplasia? Secondary hyperaldosteronism? Adenoma?

Spironolactone Tx underlying cause Surgery

What are the causes of ACTH independent Cushing syndrome?

Adrenal adenoma or carcinoma Macro nodular hyperplasia Primary pigmented nodular adrenal disease McCune-Albright syndrome

MEN2A has what tumors? Called what? Mutation in what gene?

Pheochromocytomas, medullary carcinoma of thyroid (usually AGGRESSIVE), parathyroid hyperplasia Sipple syndrome RET

What is the Diagnose of the following? ACTH Inc Dexamethasone suppression low dose = no suppression Dexamethasone suppression high dose = no suppression

Ectopic ACTH

21-H deficiency in males causes what CFs?

Enlargement of external genitalia, precocious puberty | Oligospermia in older males

What is the screening test for primary hyperaldosteronism? Confirmation test?

Elevated ratio of plasma aldosterone to renin | Aldosterone suppression test Admin saline and if aldosterone is not suppressed then confirmed primary

Familial pheochromocytomas occur more often with what? Pts?

50% b/l 25% paragangliomas Younger

What is autoimmune destruction of steroidogenic cells? Antibodies to what? Occurs in what setting?

Autoimmune adrenalitis AutoAbs to 21-H and 17-H APS1 and APS2

What presents as genital ambiguity w/progressive virilization w/1/3 of 21-H def?

Simple virilizing andreogenital syndrome w/out salt wasting

How to Dx Cushing? How to determine the cause?

Increased 24-H urine free cortisol Serum ACTH Dexamethasone suppression test: urinary excretion of 17-H after admin

What causes glucocorticoid-Remediated hyperaldosteronism? ACTH does what? Tx?

Mutated CYP11B2 on ch. 8 ACTH stimulates production of aldosterone Dexamethasone

What is the most common CAH w/only a partial def in 21-H? Sx?

Nonclassic or Late-onset adrenal virilism Asymptomatic or hirsuitism, acne, menstrual irregularity

Primary adrenocortical insufficiency is called what? Caused by what?

Addision's Progressive destruction of adrenal cortex (need 90% destruction)

What syndromes have pheochromocytomas?

MEN2A, MEN2B NF1 VHL SDHD-B

APS2 starts when? Presents how?

Early adulthood Combo of AI, autoimmune thyroiditis or T1DM

MEN1 has what tumors? Called what else? Mutations in what?

Primary hyperparathyroidism, endocrine tumors of pancreas, pituitary (PRL) Wermer Syndrome Menin protein (MLL, JunD)

Chromaffin cells are from what?

Neural crest

What are some random other causes of Addison's?

Infections (Tb and fungal, AIDS) Lung and breast carcinoma Congenital adrenal hypoplasia Adrenoleukodystrophy

Adrenocortical adenomas are what color? Weight what? Composed of what cells?

Yellow w/well-developed capsule < 30 gm Look like normal ZF

What causes Waterhouse-Friderichsen Syndrome? CFs?

N meningitidis, pseudomonas, pneumococci, H influ, staph Rapidly progressive hypotension leading to shock DIC w/purpura Massive b/l adrenal hemorrhage

What is congenital adrenal hyperplasia (CAH)? Inheritance?

Deficiency/lack of an enzyme in biosynthesis of cortical steroids (esp. cortisol) AR

What is the Diagnose of the following? ACTH Inc Dexamethasone suppression low dose = no suppression Dexamethasone suppression high dose = suppression

Pituitary Cushing

What is the most common cause of primary hyperaldosteronism? Morphology? Pt demographic?

Bilateral Idiopathic Hyperaldosteronism Bilateral nodular wedge-shaped hyperplasia of the adrenal glands Older

Autoimmune adrenalitis, hypoparathyroidism, idiopathic hypogonadism and pernicious anemia associated with what? Mutation in what? Develop autoAbs to what?

APS1 AIRE on ch. 21q22 IL-17 and IL-22 (need to fight fungus)

21-H deficiency in females causes what CFs?

Clitoral hypertrophy, pseudo hermaphroditism in infants | Oligomenorrhea, hirsutism, acne

What does primary hyperaldosteronism do to the RAAS system? Plasma renin? Most common manifestation?

Suppresses it Dec Elevated BP

Early CF of Cushing's? Later?

HTN, weight gain turn all obesity, moon facies, buffalo hump

What are the most common causes of ACTH-independent Cushing syndrome? Cortisol level? ACTH?

Adrenal adenoma and carcinoma Inc Dec

What secondary diabetic effects may occur w/glucocorticoid excess?

Hyperglycemia Glucosuria Polydipsia

What the CAH effects on the adrenal medulla?

Hypotension and circulatory collapse due to DEC catecholamines

Pts w/ familial idiopathic hyperaldosteronism have mutations in what?

KCNJ5, encodes K+ channel

What is secondary hyperaldosteronism seen in?

Dec renal perfusion Arterial hypovolemia and edema Pregnancy

What hormones are found in the adrenal medulla?

Catecholamines - Epinephrine and Norepinephrine

What is a mutation activating GNAS which causes Inc Gs, Inc cAMP and primary macronodular hyperplasia?

McCune-Albright syndrome

What is the Diagnose of the following? ACTH Dec Dexamethasone suppression low dose = no suppression Dexamethasone suppression high dose = no suppression

Adrenal tumor

Adrenocortical carcinomas are what size? What important feature?

> 200-300 gm Unencapsulated

What is composed of 1-3 mm darkly pigmented (brown/black) micronodules w/atrophic intervening areas w/Endogenous hypercortisolism? Pigment is what?

Micronodular hyperplasia Lipofuscin

What is described by adrenals almost entirely replaced by prominent nodules of varying size (< 3cm), and areas between the macroscopic nodules also demonstrate evidence of microscopic nodularity? Due to what?

Macronodular hyperplasia Endogenous hypercortisolism

The majority of ACTH-secreting pituitary adenomas are what? Account for what % of endogenous hypercortisolism? Most frequently in whom?

Microadenoma 70% Young adults

Exogenous glucocorticoids cause what morphology of adrenal glands?

Cortical atrophy, lack of stimulation of ZF and ZR

What are the causes of ACTH dependent Cushing syndrome?

Pituitary ademona | Ectopic corticotropin syndrome (ACTH)