Pheochromocytoma classification
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Pheochromocytoma Microchapters |
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Diagnosis |
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Pheochromocytoma classification On the Web |
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American Roentgen Ray Society Images of Pheochromocytoma classification |
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Risk calculators and risk factors for Pheochromocytoma classification |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2]
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Overview
Pheochromocytoma can be either benign or malignant and can be localized, regional, or metastatic.
Classification
Pheochromocytoma can be either benign or malignant and can be localized, regional, or metastatic.
Familial paraganglioma — Familial paraganglioma is an autosomal dominant disorder characterized by paragangliomas that are located most often in the skull base and neck but also in the thorax, abdomen, pelvis, and urinary bladder. (See "Pheochromocytoma in genetic disorders", section on 'Familial paraganglioma'.)
Non-familial syndromes — Pheochromocytomas and paragangliomas also are associated with syndromes that do not appear to have a familial basis. These include cholelithiasis, renal artery stenosis, and Carney's syndrome or triad (gastrointestinal stromal tumor [GIST], pulmonary chondromas, paraganglioma, adrenal cortical adenoma, and esophageal leiomyoma). The clinical features of these disorders are discussed in detail elsewhere. (See "Epidemiology, classification, clinical presentation, prognostic features, and diagnostic work-up of gastrointestinal mesenchymal neoplasms including GIST", section on 'Pediatric GIST'.)
Sporadic tumors — Even in patients with apparently sporadic pheochromocytomas, up to 25 percent will have unsuspected germline mutations of the RET, VHL, SDHD, SDHB, SHDC, SDHAF2, SDHA, TMEM127, or MAX genes [38]. Genetic testing of these patients may identify individuals and families at risk for other associated tumors. (See "Pheochromocytoma in genetic disorders", section on 'Sporadic pheochromocytoma'.)