Phaeochromocytoma/paraganglioma and adverse clinical outcomes in patients with neurofibromatosis-1.

ABSTRACT: Phaeochromocytomas/paragangliomas (PHAEO/PG) are linked to hereditary syndromes including neurofibromatosis type 1 (NF-1). Current guidelines do not recommend biochemical screening for PHAEO/PG in asymptomatic or normotensive patients with NF-1. This strategy may miss preventable morbidities in those patients who ultimately present with symptomatic PHAEO/PG. Our aim was to review the literature and extract data on mode of presentation and the incidence of reported adverse outcomes. PubMed and EMBASE literature search using the keywords "Phaeochromocytoma", "Paraganglioma" and "Neurofibromatosis" was performed looking for reported cases from 2000 to 2018. 73 reports of NF-1 patients with PHAEO/PG were found. Patients were predominately women (n=40) with a median age of 46 years (range 16-82

SUBMITTER: Al-Sharefi A 

PROVIDER: S-EPMC6198184 | biostudies-literature | 2018 Aug

REPOSITORIES: biostudies-literature