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PHEOCHROMOCYTOMA
 These tumors arising from adrenal
medulla produce, store, and secrete
catecholamines.
 Paragangliomas are tumors that
arise from autonomic ganglia and
behave pathophysiologically like
pheochromocytomas
 Most pheochromocytomas secrete
both epinephrine and
norepinephrine, with the fraction of
secreted norepinephrine being
greater than that secreted by the
normal gland.
 Most (85% to 90%) pheochromocytomas are solitary
tumors localized to a single adrenal gland, usually
the right
 Approximately 10% of adults and 25% of children
have bilateral tumors.
 Malignant spread of these highly vascular tumors
occurs in approximately 10% of cases.
 It may be part of the multiple endocrine neoplasia
(MEN) IIA or IIB.
1)Type IIA includes medullary carcinoma of the
thyroid, parathyroid hyperplasia, and
pheochromocytoma;
2) type IIB consists of medullary carcinoma of
the thyroid, pheochromocytoma, and neuromas of
the oral mucosa.
Clinical Presentation
 Most common in young to middle adult life.
 clinical manifestations are mainly due to the
pharmacologic effects of the catecholamines
released from the tumor.
 sustained hypertension maybe present although
occasionally it is paroxysmal.
 When true paroxysms occur, the blood pressure
may rise to alarmingly high levels, placing the
patient at risk for cerebrovascular hemorrhage,
heart failure, dysrhythmias, or myocardial
infarction.
 Headache, palpitations, tremor, profuse
sweating, and either pallor or flushing may
accompany an attack.
Diagnosis:
 Urinary vanillylmandelic acid and unconjugated
norepinephrine and epinephrine levels are
measured in a 24-hour urine collection and are
expressed as a function of the creatinine
clearance
 Excess production of metabolites of
catecholamines is diagnostic for
pheochromocytoma.
Anesthetic Considerations
Preoperative Preparation:
 Perioperative blood pressure fluctuations,
myocardial infarction, congestive heart failure,
cardiac dysrhythmias, and cerebral hemorrhage
all appear to be reduced in frequency when the
patient has been treated before surgery with α-
blockers and the intravascular fluid compartment
has been re-expanded.
pheochromocytoma.pptx
pheochromocytoma.pptx

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pheochromocytoma.pptx

  • 2.  These tumors arising from adrenal medulla produce, store, and secrete catecholamines.  Paragangliomas are tumors that arise from autonomic ganglia and behave pathophysiologically like pheochromocytomas  Most pheochromocytomas secrete both epinephrine and norepinephrine, with the fraction of secreted norepinephrine being greater than that secreted by the normal gland.
  • 3.  Most (85% to 90%) pheochromocytomas are solitary tumors localized to a single adrenal gland, usually the right  Approximately 10% of adults and 25% of children have bilateral tumors.  Malignant spread of these highly vascular tumors occurs in approximately 10% of cases.  It may be part of the multiple endocrine neoplasia (MEN) IIA or IIB. 1)Type IIA includes medullary carcinoma of the thyroid, parathyroid hyperplasia, and pheochromocytoma; 2) type IIB consists of medullary carcinoma of the thyroid, pheochromocytoma, and neuromas of the oral mucosa.
  • 4. Clinical Presentation  Most common in young to middle adult life.  clinical manifestations are mainly due to the pharmacologic effects of the catecholamines released from the tumor.  sustained hypertension maybe present although occasionally it is paroxysmal.  When true paroxysms occur, the blood pressure may rise to alarmingly high levels, placing the patient at risk for cerebrovascular hemorrhage, heart failure, dysrhythmias, or myocardial infarction.
  • 5.  Headache, palpitations, tremor, profuse sweating, and either pallor or flushing may accompany an attack. Diagnosis:  Urinary vanillylmandelic acid and unconjugated norepinephrine and epinephrine levels are measured in a 24-hour urine collection and are expressed as a function of the creatinine clearance  Excess production of metabolites of catecholamines is diagnostic for pheochromocytoma.
  • 6. Anesthetic Considerations Preoperative Preparation:  Perioperative blood pressure fluctuations, myocardial infarction, congestive heart failure, cardiac dysrhythmias, and cerebral hemorrhage all appear to be reduced in frequency when the patient has been treated before surgery with α- blockers and the intravascular fluid compartment has been re-expanded.