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Endo > SM_180b: Adrenal Medulla > Flashcards

SM_180b: Adrenal Medulla Flashcards

1
Q

Adrenal gland is composed of ____ and ____

A

Adrenal gland is composed of medulla and cortex

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2
Q

Adrenal cortex is derived from the ____ and secretes ____ hormones

A

Adrenal cortex is derived from the mesoderm and secretes steroid hormones

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3
Q

Adrenal medulla is derived from ____ and secretes ____

A

Adrenal medulla is derived from ectodermal chromaffin tissue and secretes catecholamines

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4
Q

Describe the development of the adrenal medulla

A

Adrenal gland medulla

  • 7 week gestation: chromaffin cells from the celiac plexus collect on the medial side of the primordial cortex and migrate inward
  • 4 months gestation: chromaffin tissue occupies a central position in a gland
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5
Q

Describe histology of the adrenal gland

A

Adrenal gland

  • Capsule
  • Zone glomerulosa
  • Zona fasciculata
  • Zona reticularis
  • Adrenal medulla
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6
Q

Adrenal medulla functions like a ____ but releases ____ into the ____

A

Adrenal medulla functions like a postganglionic sympathetic neuron but releases epinephrine into the blood stream

(receptors and effector cells located throughout body)

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7
Q

Adrenal medulla is part of the ____ system and derived from the ____

A

Adrenal medulla is part of the sympathetic nervous system and derived from the neuroectoderm (neural crest) cells that migrate from the vertebral column to the periaortic region

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8
Q

Paraganglia are ____

A

Paraganglia are sympathetic preganglionic nerve fibers that terminate in paravertebral and prevertebral nerve ganglia

  • Chromaffin cells aggregated in close approximation to autonomic ganglia and plexuses
  • (Postganglionic nerve fibers secrete norepinephrine)
  • Develop at 2 months of gestation
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9
Q

Paraganglia contain ____ cells similar to ____ cells

A

Paraganglia contain neuroendocrine cells similar to adrenal medullary cells

(catecholamines present)

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10
Q

Describe locations of the paraganglia

A

Paraganglia locations

  • Mediastinum
  • Abdomen: along sympathetic nerve chains in paravertebral and prevertebral positions
  • Around the celiac axis
  • Renal medullae
  • Aortic bifurcation (organ of Zuckerandl)
  • Adjacent to bladder
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11
Q

Pheochromocytes are ____

A

Pheochromocytes are axonless neurons producting catecholamines

  • Respond to synaptic activation by releasing preformed catecholamines: epinephrine (80%), norepinephrine (20%)
  • Regulated by sympathetic cholinergic fibers acting on nicotinic receptors
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12
Q

____ and ____ are common chromaffin cell tumors

A

Pheochromocytomas and paragangliomas are common chromaffin cell tumors

(pheochromocytomas are most common)

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13
Q

Chromogranins are ____ that can serve as ____

A

Chromogranins are chromaffin cell proteins that can serve as clinical markers (Chromogranin A)

  • Found in adrenal medulla or ganglia tumors (pheochromocytomas, paragangliomas)
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14
Q

Circulating catecholamines are metabolized in ____ and ____

A

Circulating catecholamines are metabolized in neuronal tissues and liver

  • Metabolites are excreted in the urine: 24 h urine metanephrines (catecholamine metabolites) are very valuable diagnostic tool
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15
Q

Describe catecholamine metabolite synthesis

A

Catecholamine metabolite synthesis

  • MAO degrades epinephrine and norepinephrine
  • COMT converts dihydroxymandelic acid to vanillylmandelic acid
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16
Q

Adrenal medulla secretes ____ and ____

A

Adrenal medulla secretes epinephrine and norepinephrine

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17
Q

Alpha receptors mediate ____ in vasculature and ____ in endocrine pancreas

A

Alpha receptors mediate constriction (arteries and veins) in vasculature and inhibition of secretion in endocrine pancreas

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18
Q

Beta receptors mediate ____ in vasculature and ____ in endocrine pancreas

A

Beta receptors mediate dilatation (arteries) in vasculature and stimulation of secretion in endocrine pancreas

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19
Q

Catecholamines (epinephrine and norepinephrine) are responsible for ____, ____, and ____

A

Catecholamines (epinephrine and norepinephrine) are responsible for fright, flight, and flight

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20
Q

Tumors in the adrenal medulla are ____

A

Tumors in the adrenal medulla are pheochromocytomas

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21
Q

Tumors from extra-adrenal sympathetic and parasympathetic ganglia are ____

A

Tumors from extra-adrenal sympathetic and parasympathetic ganglia are paragangliomas

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22
Q

Patients with disease-causing germline mutations are likely to have ____

A

Patients with disease-causing germline mutations are likely to have pheochromocytomas or paragangliomas

23
Q

Pheochromocytomas are tumors arising from ____ cells producing one or more ____

A

Pheochromocytomas are tumors arising from adrenomedullary chromaffin cells producing one or more catecholamines

(epinephrine, norepinephrine, and dopamine)

24
Q

Adrenal masses are most commonly ____

A

Adrenal masses are most commonly non-functioning adenomas

25
Q

Multiple endocrine neoplasia is ____ with ____ inheritance

A

Multiple endocrine neoplasia (MEN) is familial endocrine neoplasms with autosomal dominant inheritance

  • Due to gene mutations affecting the germline
  • Tumors are often bilateral or multifocal
  • Hyperplasia may occur in conjunction with neoplasia
26
Q

MEN 2A involves ____, ____, and ____

A

MEN 2A involves medullary thyroid carcinoma, pheochromocytoma, and primary hyperparathyroidism

27
Q

MEN 2B involves ____, ____, ____, ____, and ____

A

MEN 2B involves medullary thyroid carcinoma, pheochromocytoma, mucosal neuromas, GI tract ganglioneuromatosis, and marfanoid habitus

28
Q

Thin face, big lips, and nodules on the tongue are indicative of ____

A

Thin face, big lips, and nodules on the tongue are indicative of MEN 2B

29
Q

MEN 2 involves activation of ____

A

MEN 2 involves activation of RET proto-oncogene

  • RET: cell surface tyrosine kinase receptor
  • RET germline poitn mutations are found in the majority of MEN 2A and 2B patients
  • Mutations are few and clustered
  • Genotype-phenotype correlation
30
Q

RET is a ____

A

RET is a cell surface tyrosine kinase receptor

31
Q

Von Hippel Lindau Disease involves ____, ____, ____, and ____

A

Von Hippel Lindau Disease involves retinal angiomas, CNS hemangioblastomas, renal cysts / carcinomas, pancreatic cysts, pheochromocytomas

  • VZHL gene on chromosome 3 (3 exons, 284 codons)
32
Q

Pheochromocytoma in Von Hippel Lindau disease involves ___ mutations in the ___ gene

A

Pheochromocytoma in Von Hippel Lindau disease involves missense mutations in the VHL tumor suppressor gene

  • Chromosome 3p25-26
  • Encodes a protein regulating hypoxia-induced protein
  • Specific mutations associated with particular patterns of tumor formation
33
Q

Neurofibromatosis-1 is an ____ disorder characterized by ____, ____, ____, and ____

A

Neurofibromatosis-1 is an autosomal dominant disorder characterized by neurofibromas, cafe au lait spots, axillary and inguinal freckling, and iris hamartomas (Lish nodules)

34
Q

Some people with NF-1 develop ____

A

Some people with NF-1 develop catecholamine-secreting tumors

  • Solitary benign adrenal pheochromocytoma
  • Bilateral adrenal pheochromocytoma
  • Peri-adrenal abdominal paraganglioma
35
Q

Paragangliomas are characterized as ____ or ____ depending on the type of paraganglia of origin

A

Paragangliomas are characterized as sympathetic or parasympathetic depending on the type of paraganglia of origin

  • Sympathetic: arise from chromaffin cells of paraganglia along sympathetic chains in the chest, abdomen, or pelvis
  • Parasympathetic: arise from glomera along parasympathetic nerves in the head / neck / upper mediastinum
36
Q

Parasympathetic paragangliomas are usually ____ and produce ____

A

Parasympathetic paragangliomas are usually non-functional and produce no or low levels of catecholamines

37
Q

Paragangliomas usually present as ____

A

Paragangliomas usually present as slow-growing painless cellular masses

  • Many patients are asymptomatic
  • Tumor space occupation may cause pain, hearing disturbances, hoarseness, and dysphagia
38
Q

Familial paragangliomas are ____ disorders with location most commonly in the ____

A

Familial paragangliomas are autosomal dominant disorders with location most commonly in the abdomen

(second most common location in head / neck)

39
Q

Familial paragangliomas result from genetic mutations in the ____ genes

A

Familial paragangliomas result from genetic mutations in the SDH succinate dehydrogenase subunit genes

(SDHB, SDHC, SDHD)

40
Q

Succinate dehydrogenase is a component of the ____ that ____

A

Succinate dehydrogenase is a component of the mitochondrial ETC complex II that catalyzes oxidation of succinate to fumarate in the Krebs cycle

41
Q

Subunits SDHA and SDHB constitute the ____ of succinate dehydrogenase, while subunits SDHC and SDHD ____

A

Subunits SDHA and SDHB constitute the catalytic core of succinate dehydrogenase, while subunits SDHC and SDHD anchor the complex to the matrix face of the mitochondrial inner membrane

42
Q

Defects in SDHA cause ___

A

Defects in SDHA cause neurodegenerative disorders

43
Q

Mutations in SDHB, SDHC, and SDHD predispose to ____

A

Mutations in SDHB, SDHC, and SDHD predispose to familial paraganglioma syndromes (PGLI 1, 2, 3, 4)

44
Q

SDHB mutation carriers develop paraganglioma ___

A

SDHB mutation carriers develop paraganglioma early

  • More likely to develop malignant paragangliomas and additional neoplasms (e.g. renal cell carcinoma)
45
Q

Patients with sporadic pheochromocytomas / paragangliomas present at a ___ age

A

Patients with sporadic pheochromocytomas / paragangliomas present at a later age

46
Q

Headache, palpitations, diaphoresis, HTN, and abdominal pain indicate ____

A

Headache, palpitations, diaphoresis, HTN, and abdominal pain indicate pheochromocytomas / paragangliomas

47
Q

____, ____, ____, and ____ increase catecholamine levels

A

Pheochromocytomas, caffeine, marijuana, and nicotine increase catecholamine levels

48
Q

Paragangliomas are commonly associated with ____

A

Paragangliomas are commonly associated with syndromes

(such as due to SDHB mutations)

49
Q

These are ____

A

These are pheochromocytomas

50
Q

Pheochromocytomas is treated with ___ after ___

A

Pheochromocytomas is treated with surgical resection after preparation with alpha and beta blockade to control HTN and expand volume

51
Q

____ and ____ blockade is utilized to control BP and prevent intraoperative hypertensive crisis in patients with pheochromocytoma

A

Combined alpha and blockade is utilized to control BP and prevent intraoperative hypertensive crisis in patients with pheochromocytoma

52
Q

____ is given first in pharmacological preparation of pheochromocytoma

A

Phenoxybenzamine (alpha adrenergic blockade) is given first in pharmacological preparation of pheochromocytoma

  • NEVER start beta adrenergic blockers before alpha blockers b/c can further elevate BP
53
Q

High Na+ diet is used in preparation of pheochromocytoma for surgery by ____ and ____

A

High Na+ diet is used in preparation of pheochromocytoma for surgery by counteracting catecholamine induced volume contraction and orthostasis associated with alpha blockade

(> 5 g Na/day)

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