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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.
Financial & competing interest disclosures
A van Berkel receives funding from the European Union Seventh Framework Programme (FP7/2007–2013) under grant agreement no. 259735 (ENSAT CANCER). JWM Lenders receives funding from Deutsche Forschungsgemeinschaft KFO252 (WI 3660/1–1). The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.
No writing assistance was utilized in the production of this manuscript.
Notes
PGL: Paraganglioma; PHEO: Pheochromocytoma.