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Recent developments in clinical and molecular research on these tumor forms have significantly clarified their genetic backgrounds and challenged the view of \u201cpheochromocytoma as the 10% rule tumor.\u201d Firstly, a larger proportion of these tumors are today discovered in normotensive patients during imaging carried out for other reasons than suspicion of pheochromocytoma. Secondly, although the differential diagnosis between malignant and benign tumors remains a challenge, the risk of malignancy well exceeds the classical 10% in patients with extra\u2010adrenal disease, and\/or carriers of germ\u2010line SDHB mutations. Finally, up to a third of patients carry a germ\u2010line mutation in a gene predisposing to pheochromocytoma and\/or paraganglioma. Identification of a constitutional mutation in RET, VHL, SDHD, or SDHB has implications for clinical screening and follow\u2010up for both the patient and for relatives at risk who can be identified by screening for the same mutation. Genetic testing in apparently sporadic cases is therefore regarded as beneficial, especially in patients diagnosed before 50 years of age, and in patients with bilateral, multifocal, malignant and\/or extra\u2010adrenal disease. J. Surg. Oncol. 2005;89:193\u2013201. \u00a9 2005 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/jso.20177","type":"journal-article","created":{"date-parts":[[2005,2,18]],"date-time":"2005-02-18T01:49:16Z","timestamp":1108691356000},"page":"193-201","source":"Crossref","is-referenced-by-count":130,"title":["Pheochromocytoma and functional paraganglioma syndrome: No longer the 10% tumor"],"prefix":"10.1002","volume":"89","author":[{"given":"Elisabeth 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