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Endocrinology > Pheochromocytoma > Flashcards

Pheochromocytoma Flashcards

1
Q

Pheochromocytoma

-definition

A
  • chromaffin cell tumor of the adrenal cortex secreting catecholamines: adrenaline, noradrenaline, dopamine
  • tumors that develop from extra-adrenal chromaffin cells = paragangliomas, usually paravertebral
  • excessive nervous system stimulation
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2
Q

Pheochromocytoma

-prevalence (3)

A
  • mostly - sporadic
  • 1/3 - familial disorders associated with gene mutations
  • increases mortality from cardiovascular disease
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3
Q

Pheochromocytoma

-characteristic (3)

A
  • location: 95% abdomen and pelvis, paraganglioma next to aorta 75%
  • malignancy is detected only when the primary spread process is found or metastases occur –> no reliable markers
  • long term follow up is needed
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4
Q

Pheochromocytoma

-with what genetic syndrome Pheo is associated?

A

-autosomal dominant inheritance

  • MEN2 - Multiple endocrine neoplasia type 2
  • VHL - von Hippel-Lindau
  • NF1 - Neurofibromatosis type 1
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5
Q

Multiple endocrine neoplasia type 2 (MEN2)

A
  • MEN2A (Sipple’s syndrome) - 100% prevalence of medullary thyroid carcinoma (MTC), pheochromocytoma, primary hyperparathyroidism,
  • MEN2B - 100% prevalence of medullary thyroid carcinoma (MTC), pheochromocytoma, mucocutaneous neuromas, skeletal deformities
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6
Q

von Hipplel - Lindau (VHL) (6)

A
  • pheochromocytoma
  • paraganglioma
  • hemangioblastoma
  • retinal angioma
  • clear cell renal cell carcinoma
  • pancreatic neuroendocrine tumors
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7
Q

Neurofibromatosis type 1 (NF1) (7)

A
  • neurofibromas
  • multiple “cafe au lait” spots
  • iris hamartomas
  • bony abnormalities
  • CNS gliomas
  • pheochromocytoma and paraganglioma
  • macrocephaly and cognitive deficits
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8
Q

SDH - succinate dehydrogenase mutations

A

Familial pheochromocytoma/ paraganglioma

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9
Q

Pheochromocytoma

-clinics (4)

A
  • symptoms in about 50%
  • classic triad: headache, increased sweating, palpitations
  • some will have arterial hypertension

-other symptoms - less common: cardiomyopathy (takotsubo), orthostatic hypotension, visual disturbances, heart failure

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10
Q

Pheochromocytoma

-diagnostics - indications for Pheo testing (9)

A
  1. Classic symptoms
  2. Spontaneous episodes of palpitations, agitation, headaches, tremors, paleness
  3. Arterial hypertension at young age
  4. Familial syndromes
  5. Pheo in family history
  6. Adrenal incidentaloma
  7. Sudden rise in BP during anesthesia, surgery, diagnostic intervention
  8. Idiopathic dilated cardiomyopathy
  9. Former gastric stromal tumor or lung chondroma
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11
Q

Pheochromocytoma

-diagnostics - biochemical screening tests

A
  • fractionated plasma metanephrines or fractionated methanephrines at 24 hours urine
  • positive –> increase in methanephrine concentration exceeds the normal range by 2-3 times
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12
Q

Pheochromocytoma

-Chromogranin A (4)

A
  • accumulated in secretory vesicles of chromaffin adrenal cells
  • specific for neuroendocrine tumors, adrenocortical carcinoma, prostate cancer with neuroendocrine differentiation
  • amount depends on the size and spread of the tumor
  • increases –> drugs - proton pump inhibitors
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13
Q

Pheochromocytoma

-Dopamine

A
  • rarely pheochromocytomas secrete dopamine alone

- test –> 3 methoxytiramine or plasma dopamine

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14
Q

Pheochromocytoma

-radiological testing (5)

A
  • recommended only with biochemical confirmation of Pheo
  • CT -first choice
  • MRI - for suspected metastasis
  • Scintigraphy with J123 MIBG - for patients with or with risk of metastasis, when the tumor is outside the adrenal gland (paraganglioma), in case of relapse
  • PET - for patients with known metastasis, more sensitive than Scintigraphy
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15
Q

Pheochromocytoma

  1. Pheo at CT: (5)
  2. Pheo at MRI:
A
  1. large tumor (usually >3cm), hemorrhages, inhomogeneous, mostly >10 HU density, calcification is rare
  2. T1: hypo intense or isointense mass , T2: high-intensity mass
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16
Q

Pheochromocytoma

-genetic testing

A
  • last step

- all patients who are diagnosed with Pheo or paraganglioma, should be referred for genetic counseling

17
Q

Pheochromocytoma

-Associated genetic syndromes - after diagnosis of Pheo also investigate: (4)

A
  1. PTH and ionized calcium for primary hyperparathyroidism (MEN2A)
  2. Calcitonin for medullary thyroid carcinoma (MEN2A)
  3. Ophthalmologists consultation to evaluate retina for angiomas (VHL)
  4. Radiological examination to evaluate kidneys and pancreas for cysts (VHL)
18
Q

Pheochromocytoma

-treatment (3)

A
  • surgical
  • imp. –> preoperative patient preparation
  • medication for receptor blockade before surgery –> first-line: alpha-adrenoblockers (AAB)
19
Q

Pheochromocytoma

-treatment - preparation scheme (6)

A
  1. alpha-adrenoblockers (AAB) are started 7-14 days prior to surgery
  2. if tachycardia –> add betablockers, not earlier than 3-4 days after AAB
  3. if high BP persists –> add calcium blockers
  4. if high BP persists –> add methyrosine
  • ensure adequate salt >5 g/d and fluids intake
  • 1-2 days before surgery –> administer crystalloid infusions
20
Q

Pheochromocytoma

  • preparation scheme:
    1. Target BP
    2. Target heart rate
A
  1. BP <130/80 mmHg sitting and diastolic >90 mmHg standing (range depends on patient age and comorbidities)
  2. HR - 60-80 t/min
21
Q

Pheochromocytoma

-surgical treatment (4)

A
  • Laparoscopy - first choice - solitary adrenal Pheo (tumor size <6cm)
  • Laparotomy - locally invasive or malignant Pheo and paragangliomas - size >6cm
  • Bilateral adrenalectomy - patients diagnosed with MEN2 syndrome
  • Adrenal cortex sparing adrenalectomy - when Pheo is very small
22
Q

Pheochromocytoma

-long-term follow up (3)

A
  • 14-28d. after surgery –> measure fractionated metanephrines –> if increased –> look for metastasis
  • long-term follow up –> once a year
  • follow up duration –> 10 years for all –> high risk patients (young age, large mass, paraganglioma, genetic disease) –> whole life
23
Q

Pheochromocytoma

-factors that may influence the results (4)

A
  • the blood is drawn in the morning, on an empty stomach, patient must lie on the back
  • food, coffee, alcohol or smoking 12 hours prior –> not allowed
  • conditions causing sympathetic nervous system activation and promoting catecholamines secretion (aka stress conditions): stroke, MI, obstructive sleep apnea, congestive heart failure, hypoglycemia, increased intracranial pressure, hypoxia, acidosis, surgery, trauma –> up to 10 days after the event
  • drugs –> if possible, discontinue at least 2 weeks before hormone testing

Endocrinology (11 decks)

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