EPCephalic duodenopancreatectomy for neurofibromatosis associated with gastrointestinal stromal tumor. A case report
Florin Graur 1, Vlad I. Nechita 2, Sorana D. Bolboacă 3, Mihaela Dragotă 4, Ana M. Cosma 4, Nadim AI Hajjar 5
1 Department of Surgery, Faculty of Medicine, “Iuliu Hațieganu” University of Medicine and Pharmacy, Romania
2 “Prof. Dr. Octavian Fodor” Regional Institute of Gastroenterology and Hepatology, Surgery Department Cluj-Napoca, Romania； Department of Medical Informatics and Biostatistics, Faculty of Medicine, “Iuliu Hațieganu” University of Medicine and Pharmacy, Cluj-Napoca, Romania
3 Department of Medical Informatics and Biostatistics, Faculty of Medicine, “Iuliu Hațieganu” University of Medicine and Pharmacy, Cluj-Napoca, Romania
4 “Prof. Dr. Octavian Fodor” Regional Institute of Gastroenterology and Hepatology, Surgery Department Cluj-Napoca, Romania
5 “Prof. Dr. Octavian Fodor” Regional Institute of Gastroenterology and Hepatology, Surgery Department Cluj-Napoca, Romania；Department of Medical Informatics and Biostatistics, Faculty of Medicine, “Iuliu Hațieganu” University of Medicine and Pharmacy, Cluj-Napoca, Romania
Ann. Ital. Chir. 2019, 8(June), 1–5
Published: 13 Jun 2019
Abstract
BACKGROUND: Neurofibromatosis is a genetic autosomal dominant disease characterized by multiple skin nodules and hyperpigmentation. This condition is frequently associated with a large variety of neoplasia, including gastrointestinal stromal tumors (GIST) in about 6% of cases. We present a case of neurofibromatosis associated GIST. CASE REPORT: A 57 year-old male patient with Von Recklinghausen disease was referred into our service after he was discovered with a 7 mm nodular formation in contact with the pancreatic head, during a routine abdominal ultrasonography. Ultrasound examination performed into our service reveals a nodular formation with hypoechoic circumference, central translucency, with central necrosis, contrast medium uptake and dimensions at about 78/49/77 mm, without peritoneal fluid content. Trans-gastric biopsy specimen shows mesenchymal proliferation with spindle cells and elongated nuclei and c-kit intensely positive. The diagnosis was gastrointestinal stromal tumor. Delimitation towards pancreatic head was unclear. A cephalic duodenopacreatectomy was performed. Morphopathology confirmed a multifocal GIST of low grade G1, in the second stage. CONCLUSION: A rare case of neurofibromatosis associated GIST with multifocal localization was successfully treated by cephalic duodenopancreatectomy.
