Extra-adrenal pheochromocytoma: diagnosis and management

Extra-adrenal pheochromocytomas (EAPs) may arise in any portion of the paraganglion system, though they most commonly occur below the diaphragm, frequently in the organ of Zuckerkandl. EAPs probably represent at least 15% of adult and 30% of childhood pheochromocytomas, as opposed to the traditional...

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Bibliographic Details
Published in:Current urology reports 2007-01, Vol.8 (1), p.83-88
Main Authors: Disick, Grant I S, Palese, Michael A
Format: Article
Language:English
Subjects:
Adolescent
Adrenal Gland Neoplasms - diagnosis
Adrenal Gland Neoplasms - mortality
Adrenal Gland Neoplasms - surgery
Adrenalectomy - methods
Adult
Child
Female
Follow-Up Studies
Humans
Laparoscopy - methods
Magnetic Resonance Imaging
Male
Neoplasm Staging
Pheochromocytoma - diagnosis
Pheochromocytoma - mortality
Pheochromocytoma - surgery
Risk Assessment
Survival Rate
Tomography, X-Ray Computed
Treatment Outcome
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Summary:Extra-adrenal pheochromocytomas (EAPs) may arise in any portion of the paraganglion system, though they most commonly occur below the diaphragm, frequently in the organ of Zuckerkandl. EAPs probably represent at least 15% of adult and 30% of childhood pheochromocytomas, as opposed to the traditional teaching that 10% of all pheochromocytomas are at extra-adrenal sites. They may be malignant in up to 40% of the cases, though conflicting data add to the uncertainty of this point. Patients with EAPs may present with headache, palpitations, sweating, or hypertension. A small percent of patients may also be asymptomatic at presentation due to nonfunctional tumors. The diagnosis is confirmed by demonstrating elevated blood and urine levels of catecholamines and their metabolites. Imaging studies to evaluate for EAPs include CT, MRI, and (131)I-labelled metaiodobenzylguanidine scintigraphy. Preoperative pharmacologic preparation, attentive intraoperative monitoring, and aggressive surgical therapy have important roles in achieving successful outcomes. Recent reports suggest that a laparoscopic approach, along with intraoperative ultrasound, can safely remove these tumors. EAPs recur and metastasize more often than their adrenal counterparts, making lifelong follow-up essential.
ISSN:1527-2737
1534-6285
DOI:10.1007/s11934-007-0025-5