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ENDO > 179/180 Adrenal > Flashcards

179/180 Adrenal Flashcards

1
Q

What are the three zones of the adrenal cortex (outside to inside)?

A

(outside) zona glomerulosa, zona fasciculata, zona reticularis

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2
Q

Cells in the zona glomerulosa…

produce:

look like on histology:

A

Cells in the zona glomerulosa…

produce: mineralocorticoids

look like on histology: clustered, high nuclear to cytoplasm ratio, pink cytoplasm, finely vaculated

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3
Q

Cells in the zona fasciculata…

produce:

look like on histology:

A

Cells in the zona fasciculata…

produce: glucocorticoids

look like on histology: arranged in columns perpendicular to capsule

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4
Q

Cells in the zona reticularis…

produce:

look like on histology:

A

Cells in the zona reticularis…

produce: androgens

look like on histology: haphazardly arranged, appear smaller, granular and pink cytoplasm

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5
Q

What are adrenal cortical heterotopia and accessory adrenal glands?

A

normal adrenal cortical in abnormal places

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6
Q

What is adrenal cortical hyperplasia? What are the three patterns?

A

bilateral increase in adrenal cortical mass due to documented endocrine abnormality

three patterns: diffuse (congenital adrenal hyperplasia, cushing syndrome), micronodular (cushing syndrome), and macronodular (rare)

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7
Q

What type of neoplasm is a unilateral solitary mass that is bright yellow in color and usually > 2 cm?

A

adrenal cortical adenoma

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8
Q

What are the microscopic pathological findings of adrenal cortical adenoma?

A

mixed growth pattern, cells with vesicular/vacuolated clear cytoplasm, can have nuclear pleomorphism

do not typically see mitoses and necrosis

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9
Q

Can you determine if a tumor is functional or non-functional from morphology?

A

generally no, it is not possible to tell based on morphology alone

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10
Q

In an adult patient, > ______ grams and > _______ cm favors an adrenal cortical carcinoma.

In a pediatric patient, > ______ grams and > _______ cm favors an adrenal cortical carcinoma.

A

In an adult patient, > 100 grams and > 6.5 cm favors an adrenal cortical carcinoma.

In a pediatric patient, > 400 grams and > 10.5 cm favors an adrenal cortical carcinoma.

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11
Q

What type of adrenal neoplasm is large and bulky with a red-brown to yellow color, hemorrhage, and necrosis?

A

adrenal cortical carcinoma

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12
Q

What is the microscopic pathology of adrenal cortical carcinoma? What criteria are used to determine malignancy?

A

highly cellular tumors in variable patterns, increased nuclear to cytoplasmic ratio, clear to pink cytoplasm, mitotic activity and atypical mitoses present

use Weiss criteria to determine malignant potential

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13
Q

An example of primary acute adrenal insufficiency is ___________, which is characterized by _____________.

A

An example of primary acute adrenal insufficiency is Waterhouse-Friderichsen Syndrome, which is characterized by hypotension, shock, and purpura of skin following an overwhelming bacterial infection (ex. Neisseria meningitidis).

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14
Q

What type of adrenal insufficiency is associated with neisseria meningitidis infection?

A

primary (acute) insufficiency –> Waterhouse-Friderichsen Syndrome

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15
Q

An example of primary chronic adrenocortical insufficiency is ____________, which is characterized by ______________.

A

An example of primary chronic adrenocortical insufficiency is Autoimmune adrenalitis like Addison’s disease, which is characterized by autoantibodies against cortical cells.

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16
Q

An example of secondary adrenal insufficiency is ____________ that often arises from ______________ (3).

A

An example of secondary adrenal insufficiency is metastatic carcinoma that often arises from breast, lung, kidney.

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17
Q

What is the main cell type of the mature adrenal medulla? What do they produce? What do they look like?

A

pheochromocytes (aka chromaffin cells)

produces catecholamines

blue cytoplasm, large, salt and pepper chromatin, prominent nucleoli

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18
Q

What is a pheochromocytoma?

A

catecholamine secreting tumor arising from adrenal medulla composed of neoplastic pheochromocytes (chromaffin cells)

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19
Q

What is the difference between a paraganglioma and a pheochromocytoma?

A

paragangliomas are catecholamine secreting tumors that are outside of the adrenal medulla (histologically the same as pheochromocytoma)

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20
Q

What are the 10% rules (5) that characterize pheochromocytomas?

A

10% bilateral

10% paragangliomas

10% in children

10% are malignant

10% are associated with familial syndromes

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21
Q

What is the gross and microscopic pathology of pheochromocytomas?

A

gross: solitary, unilateral masses, well-circumscribed, gray/white mass, large
microscopic: nested (zellballen) growth pattern, richly vascular, blue to purple cytoplasm, “salt and pepper” nuclei, rare mitoses

22
Q

What is a ganglioneuroma? What is their pathology?

A

benign tumor of neural crest derivation composed of mature ganglion cells and schwannian stroma

gross: solitary, unilateral mass, may be calcified, well-circumscribed, firm, tan-yellow, large
microscopic: mature schwannian stroma and interspersed ganglion cells

23
Q

What is a neuroblastoma? What metabolites are detectable in urine?

A

malignant tumor of neural crest derived neuroblastic cells

can detect catecholamine metabolites (VMA, HVA) in urine

24
Q

What is the pathology of neuroblastomas?

A

gross: large, firm, gray/white, hemorrhage and necrosis, cystic changes
microscopic: small cells with hyperchromatic nuclei, very little cytoplasm, high mitotic rate, abundant nuclear debris, “small round blue tumor, neuropil in stroma, homer-wright pseudo-rosettes

25
Q

What is this?

A

pheochromocytoma

  • grossly pink, red, or gray*
  • microscopically form nests (zellballen)*
26
Q

What is this?

A

neuroblastoma

homer-wright pseudo-rosettes, little blue cells

27
Q

What is the relationship between the adrenal medulla and the autonomic nervous system?

A

parts of the adrenal medulla (derived from neuroectoderm) produce epinephrine that is released into the blood stream

28
Q

What are paraganglia?

A

sympathetic pre-ganglionic nerve fibers that terminate in paravertebral and prevertebral nerve ganglia

29
Q

When do paraganglia arise in the embryo? What are their major cellular components?

A

start forming after 2 months of gestation

contain chromaffin cells (neuroendocrine)

30
Q

What are chromaffin bodies?

A

chromaffin masses arising along the course of the aorta made up of chromaffin cells and connective tissues

31
Q

What chromaffin marker is used to assess for adrenal medulla or ganglia tumors?

A

chromogranins

32
Q

Catecholamine synthesis starts with _________ that becomes oxidized to ___________ via ___________.

A

Catecholamine synthesis starts with tyrosine that becomes oxidized to L-DOPA via tyrosine hydroxylase.

33
Q

How does norepinephrine become epinephrine?

A

an enzyme PNMT methylates it (with an S-adenosyl methionine cofactor that donates a methyl group)

34
Q

What are the systemic effects of epinephrine and norepinephrine?

A

epinephrine: increased systemic BP, increased HR, decreased gut motility, circulation diverted to limb muscles, bronchodilation, mydriasis, hyperglycemia
norepinephrine: increased systolic and diastolic BP, decreased HR, hyperglycemia

35
Q

What are the two major types of adrenal medulla tumors?

A

in the adrenal medulla proper –> pheochromocytoma

in the extra adrenal sympathetic ganglia –> paragangliomas

36
Q

What are the major symptoms of MEN 2A?

A

medullary thyroid carcinoma (most common)

primary hyperparathyroidism

pheochromocytoma

37
Q

What are the major symptoms of MEN 2B?

A

medullary thyroid carcinoma, pheochromocytoma, mucosal neuromas, GI tract ganglioneuromatosis, marfanoid habitus

38
Q

What is the genetic basis of MEN 2?

A

activation of the RET proto-oncogene (a cell surface tyrosine kinase receptor)

39
Q

What types of tumors are associated with von hippel lindau disease?

A

retinal angiomas, CNS hemangioblastomas, renal cysts, renal cell carcinomas, pancreatic cysts, pheochromocytomas

40
Q

Where is the von hippel lindau gene located? What kind of gene is it?

A

chromosome 3

tumor suppressor gene that encodes a protein regulating hypoxia-induced proteins

41
Q

What features are associated with neurofibromatosis type 1?

A

neurofibromas, cafe au lait spots, axillary/inguinal freckling, iris hamartomas, rarely pheochromocytomas

42
Q

Where are most paragangliomas located?

A

abdomen

43
Q

What genetic syndromes are associated with paragangliomas?

A

mutations in succinate dehydrogenase gene (SDHB, C, and D)

44
Q

What are the symptoms of pheochromocytomas?

A

headaches, palpitations, diaphoresis, hypertension, abdominal pain, insulin resistance

could also be asymptomatic

45
Q

What are tests available for pheochromocytomas?

A

24-hour urine collection for fractionated catecholamines

24-hour urine collection for fractionated metanephrines

plasma free metanephrines (negative predicatve value)

46
Q

What is the treatment for pheochromocytomas?

A

surgical resection (usually laproscopic)

pre-surgical prep with alpha and beta blockade to prevent hypertensive crisis

47
Q

What are the necessary pre-op steps for pheochromocytoma management?

A

alpha (given first) and beta blockade to control blood pressure and prevent intraoperative hypertension

also start a high sodium diet

48
Q

Why does it matter if beta or alpha blockers are given first in patients with pheochromocytoma before surgery?

A

beta blockers lead to blockade of vasodilatory peripheral beta-adrenergic receptors (which further increases blood pressure)

alpha-adrenergic receptor stimulation must be given first to normalize blood pressure and expand blood volume

49
Q

A 21 yo woman presents with renal masses, adrenal masses, and brain hemangioblastomas. What is her most likely diagnosis?

a) pheochromocytoma
b) MEN 2B
c) paraganglioma
d) Von-Hippel Lindau
e) neurofibromatosis 1

A

d) Von-Hippel Lindau

the combination of renal masses, adrenal masses, and hemangiomas is consistent with VHL

50
Q

A 55 yo man has an extra adrenal pheochromocytoma. What is the most likely abnormal hormone level in this patient?

a) Epinephrine
b) DOPA
c) Norepinephrine
d) Calcitonin
e) Parathyroid hormone

A

c) Norepinephrine

paragangliomas usually secrete norepinephrine; the enzyme phenylethanolamine N-methyltransferase (PNMT) is present only in the adrenal medulla, not the paraganglia

51
Q

A 15 yo boy presents with MEN2B. What of the findings below are not consistent with MEN2B?

a) hyperparathyroidism
b) marfanoid habitus
c) medullary thyroid cancer
d) mucosal neuromas
e) digestive problems

A

a) hyperparathyroidism

hyperparathyroidism is consistent with MEN2B

ENDO (19 decks)

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