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Key Features

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  • Uncommon; however, up to 10% of reported cases occur in pediatric patients

  • Tumor can be located wherever chromaffin tissue (adrenal medulla, sympathetic ganglia, or carotid body) is present

  • May be multiple, recurrent, and sometimes malignant

  • Familial forms include pheochromocytomas associated with the dominantly inherited neurofibromatosis type 1, multiple endocrine neoplasia type 2, and von Hippel-Lindau syndromes, as well as mutations of the succinate dehydrogenase genes

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Clinical Findings

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  • Headache

  • Sweating

  • Tachycardia

  • Hypertension

  • Vasomotor instability (flushing and postural hypotension)

  • Anxiety

  • Dizziness, weakness

  • Nausea, vomiting, diarrhea

  • Dilated pupils, blurred vision

  • Abdominal and precordial pain

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Diagnosis

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  • Serum and urine catecholamines are elevated

  • Plasma-free metanephrine is the most sensitive and specific test, although phenoxybenzamine, tricyclic antidepressants, and β-adrenoreceptor blockers can cause false-positive results

  • Levels three times the normal range are diagnostic

  • Intermediate values may require additional testing with serum and urine catecholamines

  • After demonstrating a tumor biochemically, imaging methods including CT or MRI are used to localize the tumor

  • Nuclear medicine using functional ligands such as (123) I-MIBG, [18F]DA positron emission tomography scanning, and somatostatin receptor scintigraphy (with either [123 I]Tyr3-octreotide or [111 In] DTPA-octreotide) are useful in further diagnostic evaluation

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Treatment

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  • Laparoscopic tumor removal is the treatment of choice; however, the procedure must be undertaken with great caution and with the patient properly stabilized

  • Oral phenoxybenzamine or intravenous phentolamine is used preoperatively

  • Profound hypotension may occur as the tumor is removed but may be controlled with an infusion of norepinephrine, which may have to be continued for 1–2 days

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