Pheochromocytoma. (November 2014)

• Record Type: Journal Article
• Authors: Berkel, Anouk van; Lenders, Jacques WM
• Is Part Of: International journal of endocrine oncology. Volume 1:Number 2(2014)
• Page Start: 183
• Page End: 194
• Publication Date: 2014-11
• Subjects: catecholamines -- metanephrines -- paraganglioma -- pheochromocytoma; Endocrine glands -- Cancer -- Periodicals; 616.9944005
• DOI: 10.2217/ije.14.17
• Languages: English
• ISSNs: 2045-0869

Abstract

Pheochromocytoma and paraganglioma are catecholamine-producing tumors that are associated with substantial serious morbidity and mortality. A carefully taken medical history and early consideration of such tumors are key for early diagnosis and treatment. The biochemical diagnosis should include measurements of metanephrines in either plasma or urine. In addition to anatomical imaging, sensitive functional imaging modalities offer extra benefit for most patients in locating the tumor and eventual metastases. In at least 30% of all patients with a pheochromocytoma/paraganglioma, an underlying germline is responsible for tumor development and genetic testing should be considered in all patients. Current treatment options for malignant pheochromocytoma/paraganglioma are still very modest. New developments in the therapeutic armamentarium may improve the prospect for these patients.