Triple malignancy (NET, GIST and pheochromocytoma) as a first manifestation of neurofibromatosis type-1 in an adult patient

• DOI: 10.1186/s13000-019-0848-7
• Publication Date: 2019-07-13T13:02:44Z
• Authors: Karolina Poredska; Lumir Kunovsky; Vladimir Prochazka; Jiri Dolina; Miroslava Chovancova; Jakub Vlazny; Tomas Andrasina; Michal Eid; Petr Jabandziev; Petr Kysela; Zdenek Kala
• Keywords: Pathognomonic; Neurofibromin 1

Neurofibromatosis type-1 (NF1), also called von Recklinghausen disease, is a rare genetic disease which can lead to the development of benign or even malignant tumors. NF1 mostly diagnosed in children early adolescents who present with clinical symptoms. A curative therapy still missing and management based on careful surveillance. Concerning tumors affect gastrointestinal tract patients NF1, most common stromal tumor (GIST).