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Endocrine > 170. Adrenal Medulla Pathophysiology > Flashcards

170. Adrenal Medulla Pathophysiology Flashcards

1
Q

Enzyme that creates EPI from NE

Only found in the adrenal medulla

A

PNMT

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2
Q

Start a ___ to counteract catecholamine-induced volume contraction and orthostasis associated w/ alpha blockade

A

High sodium diet

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3
Q

Autosomal dominant disorder w/:

  • neurofibromas
  • Cafe au lait spots
  • axillary and inguinal freckling
  • iris hamartomas (lish nodules)

2% of patients develop pheochromocytomas

A

NF1

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4
Q

Proteins that can serve as clinical markers of adrenal medulla or ganglia tumors

Contained within the chromaffin cells within the paraganglia

A

Chromogranins

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5
Q

Includes:

  • Medullary thyroid carcinoma
  • Pheochromocytoma
  • Mucosal neuromas
  • GI tract ganglioneurosis
  • marfanoid habitus
  • Peculiar facial features: thin face, big lips, nodules on tongue
A

MEN2B

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6
Q

Arise from the glomera along parasympathetic nerves in the head, neck, and upper mediastinum

Clinically non-functional and produce low levels or no catecholamines

A

Parasympathetic paragangliomas

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7
Q

Tumors arising in the adrenal medulla

A

Pheochromocytoma

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8
Q

Surgical resection

Patients need to be prepared with alpha and beta blockade

A

Treatment of pheochromocytoma

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9
Q

Result of the activation of RET proto-oncogene

  • cell surface tyrosine kinase receptor RET germ-line point mutations are found in the majority
A

MEN2

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10
Q

Presentation is mostly age-dependent

Gene is located on chromosome 3

  • tumor suppressor gene

Pheochromocytoma associated w/ missense mutation

A

Von Hippel Landau Disease

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11
Q

Clinical presentation:

  • HA (71%)
  • Palpitations (65%)
  • Diaphoresis (65%)
  • HTN
  • Abdominal pain
  • Asymptomatic (10%)
  • insulin resistance state
A

Pheochromocytoma

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12
Q

Should never be started first because blockade of vasodilatory peripheral beta-adrenergic receptors with unopposed alpha-adrenergic receptor stimulation can lead to further elevation in BP

A

Beta-blockade

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13
Q

Familial paragangliomas are associated to genetic mutations in ___

Component of the ETC complex II

Catalyzes the oxidation of succinate to fumarate

A

Succinate DH

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14
Q

Given first in preoperative medical therapy for pheo resection

Given 7-10 days preoperatively to normalize BP and expand the contracted blood volume

A

Alpha-adenergic blockade (phenoxybenzamine)

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15
Q

Autosomal dominant gene mutations affecting the germ-line

  • syndrome of familial endocrine neoplasms

Two types: A and B

A

MEN 2

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16
Q

Consist of chromaffin cells that collect in close approximation to autonomic ganglia and plexuses

Found in the mediastinum, abdomen, around celiac axis, renal medullae, aortic bifurcation and adjacent to bladder

A

Paraganglia

17
Q

Can be sporadic and familial (AD disorders)

Locations:

  • head and neck (5%)
  • thorax
  • Abdomen (50%)
  • pelvis
  • bladder
A

Paragangliomas

18
Q

Secretes calcitonin Most common component of MEN2 w/ a 90% penetrance

A

Medullary thyroid carcinoma

19
Q

Most common of the MEN2 syndromes

Includes:

  • Medullary thyroid carcinoma is the most common with over 90% penetrance
  • Primary hyperparathyroidism
  • Pheochromocytoma (50%)
A

MEN2A

20
Q

Associated with families with abdominal, pelvic, and thoracic catecholamine-secreting familial paraganglioma and have the greatest risk of malignancy

Carriers develop disease early (~34yo)

More likely to develop malignant paragangliomas and additional neoplasms (renal cell carcinoma)

A

SDHB mutation

21
Q

Syndrome that includes multiple tumors:

  • retinal angiomas
  • CNS hemangioblastomas
  • Renal cysts, renal cell carcinomas
  • pancreatic cysts
  • pheochromoctyomas (25%)
A

Von Hippel Landau Disease

22
Q

Catecholamine-producing tumor of chromaffin cells

Usually intra-abdominal <0.2% of hypertension

Occasionally bilateral (10%)

Associated w/ inherited diseases:

  • MEN2A
  • MEN2B
  • Von Hippel-Lindau
  • NF1
A

Pheochromocytoma

23
Q

Target of preoperative medical therapy before pheo removal

A

Control hypertension and expand volume

24
Q

Causes the following:

  • Increase in systolic and diastolic BP
  • decrease of HR
  • hyperglycemia
A

NE

25
Q

Causes the following:

  • increase in systemic BP
  • increase in HR
  • decrease of gut motility
  • diversion of circulation to limb muscle
  • bronchodilation
  • mydriasis
  • hyperglycemia
A

EPI

26
Q

Tumors arising from the extra-adrenal sympathetic ganglia

A

Paragangliomas

27
Q

Measures metanephrine and normetanephrine in a 24 hr collection for ___

A

Fractionated metanephrines

28
Q

Variants include:

  • Familial MTC (FMTC)
  • w/ cutaneous lichen amyloidosis (CLA)
  • w/ Hirshsprung’s disease
A

MEN2A

29
Q

Arise from chromaffin cells of paraganglia along sympathetic chains in the chest, abdomen, or pelvis

A

Sympathetic paragangliomas

30
Q

Sympathetic pre-ganglionic nerve fiber that terminates in para-vertebral and pre-vertebral nerve ganglia

Postganglionic nerve fibers secrete NE

A

Paraganglia

31
Q

Measures EPI and NE in a 24hr collection for __

A

Fractionated catecholamines

32
Q

Has both an embryologic and functional link with the autonomic nervous system

  • part of the autonomic/sympathetic nervous system

Functions much like the postganglionic sympathetic neurons but epinephrine is released into the blood stream, rather than the synaptic junction

A

Adrenal medulla

33
Q

Has a negative predictive value 100%

A

Plasma free metanephrines

Endocrine (26 decks)

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