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Cited by 143Adrenal and Paraganglionic TumorsNeuroendocrine Tumor Research AdvancesHormonal Regulation and Hypertension

Pheochromocytoma

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Manger, William M., Gifford, Ray W.·2002·The Journal of Clinical Hypertension
DOI 10.1111/j.1524-6175.2002.01452.xPMC PMC8099329
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Abstract

Pheochromocytoma, a relatively rare (<0.05% of hypertensives), catecholamine-secreting tumor, is almost always lethal unless recognized and appropriately treated. Clinical and biochemical manifestations are mainly caused by excess circulating catecholamines and hypertension. Manifestations mimic many conditions, which may result in erroneous diagnoses and improper treatment. Sustained or paroxysmal hypertension associated with headaches, sweating, or palpitations, occurs in 95% of patients, but at least 5% are normotensive. All patients with manifestations of hypercatecholaminemia or coexisting neoplasms should be investigated for pheochromocytoma. Plasma free metanephrines and fractionated urinary metanephrines are the most sensitive (about 100%) chemical tests for diagnosing sporadic and familial pheochromocytomas; plasma and urinary catecholamines and total metanephrines are fairly sensitive for identifying sporadic cases but are less sensitive for familial tumors. The clonidine suppression test helps exclude other conditions that may elevate plasma and urinary catecholamines and their metabolites. Magnetic resonance imaging is more sensitive than computed tomography for localizing pheochromocytomas; iodine-131-metaiodobenzylguanidine (131I-MIBG) tumor uptake confers specificity. Surgical resection is successful in 90% of cases, but 10% of tumors are malignant. Pheochromocytomas <5 cm in diameter can be removed laparoscopically; larger tumors should be removed by open surgery. Drug treatment prior to and during surgery is mandatory; drug treatment, chemotherapy, and radiation therapy are used to treat malignant lesions.

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  • Hypertension is a common sign of pheochromocytoma, but 5% to 15% of patients may have normal blood pressure at presentation, particularly those with incidentaloma or familial screening.

    “Hypertension is a common sign of pheochromocytoma, but 5% to 15% of patients may have normal blood pressure at presentation, particularly in those with incidentaloma or having familial screening where the tumor may be smaller and less functionally active at presentation (190).”

    From Adrenal incidentaloma (2020) · cites this paper as [190]

  • Headache occurs in up to 90% of symptomatic patients with pheochromocytoma, with sweating in 60% to 70%.

    “Headache occurs in up to 90% of symptomatic patients (190) with sweating in 60% to 70%.”

    From Adrenal incidentaloma (2020) · cites this paper as [190]

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Authors
Manger, W. M., & Gifford, R. W.
Affiliations
Cleveland Clinic (US)
Corresponding author
Manger, William M.
Venue
The Journal of Clinical Hypertension
Publisher
Wiley
Published
2002-01
Volume
4
Issue
1
Pages
62-72
ISSN
  • 1524-6175 (ISSN-L)
  • 1751-7176
DOI
10.1111/j.1524-6175.2002.01452.x
PMC
PMC8099329
OpenAlex
W3188240819
Cited by
143
References
40
Citations by year
2026: 6 · 2025: 2 · 2024: 10 · 2023: 10 · 2022: 14 · 2021: 11 · 2020: 6 · 2019: 2 · 2018: 6 · 2017: 4 · 2016: 3 · 2015: 8 · 2014: 7 · 2013: 5 · 2012: 4
FWCI
1.24
Type
journal article
Language
en
Open access
Licence
onlinelibrary.wiley.com/termsAndConditions#vor (vor, 1976 d)
Locations
  • Journal of Clinical Hypertension (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
  • Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Hormonal Regulation and Hypertension
Keywords
pheochromocytoma, catecholamine-secreting tumors, hypertension, hypercatecholaminemia, metanephrine, clonidine suppression test, 131I-MIBG scintigraphy, magnetic resonance imaging, laparoscopic resection, malignant pheochromocytoma, familial pheochromocytoma
MeSH terms
Adrenal Gland Neoplasms, Algorithms, Diagnosis, Differential, Humans, Hypertension, Pheochromocytoma

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