Multiple Stromal Tumors in Von Recklinghausen Disease: A Real Diagnostic and Therapeutic Challenge

Nacir Oussama, Ait Errami Adil, Oubaha Sofia, Samlani Zouhour, Krati Khadija

Abstract: Gastrointestinal stromal tumours (GIST) are rare. They usually occur sporadically, or may be part of a pathological framework such as Neurofibromatosis type 1, and Carney's triad. Familial forms exist. GISTs associated with Neurofibromatosis Type 1 differ from sporadic GISTs in terms of epidemiology, clinical, immunohistochemical, molecular biology, prognosis, and therapeutic management. We report the case of a patient with multiple GISTs in relation to Neurofibromatosis type 1, with the aim of outlining the diagnostic and therapeutic difficulties through a review of the literature.

Keywords: Gastrointestinal stromal tumours, Von Recklinghausen disease, therapeutic challenge