Phaeochromocytoma

Screening is indicated in asymptomatic patients with an inherited disorder associated with an increased risk of phaeochromocytomas such as multiple endocrine neoplasia (MEN) 2A and 2B, Von Hippel-Lindau disease, and neurofibromatosis type 1. In the case of MEN syndromes, phaeochromocytomas develop in up to 50% of cases. The preferred screening test is serum metanephrines (also known as metadrenalines) and normetanephrines (also known as normetadrenaline) due to the high sensitivity of this test.[96]Lenders JW, Pacak K, Walther MM, et al. Biochemical diagnosis of pheochromocytoma: which test is best? JAMA. 2002 Mar 20;287(11):1427-34. https://jamanetwork.com/journals/jama/fullarticle/194752 http://www.ncbi.nlm.nih.gov/pubmed/11903030?tool=bestpractice.com ​ Patients with a personal history of a phaeochromocytoma need at least annual screening for up to 10 years after resection.[97]Huang KH, Chung SD, Chen SC, et al. Clinical and pathological data of 10 malignant pheochromocytomas: long-term follow up in a single institution. Int J Urol. 2007 Mar;14(3):181-5. http://www.ncbi.nlm.nih.gov/pubmed/17430251?tool=bestpractice.com