Long-Term Follow-Up of Duodenal Papillary Somatostatinoma Treated by Endoscopic Papillectomy

Abstract

Somatostatinomas are rare neuroendocrine tumors usually associated with Von Recklinghausen's disease, multiple endocrine neoplasia, or Von Hippel-Lindau syndrome. Nevertheless, it may be associated with sporadic occurrence in healthy patients (1) . Approximately 55% of somatostatinomas are pancreatic tumors and the remainder arises in ampullary and periampullary region or, rarely, in jejunum (2) . A patient with pancreatic somatostatinoma usually presents a triad of steatorrhea, weight loss and diabetes mellitus, due to inhibition of gastrointestinal peptides (1,3) . In duodenal papillary somatostatinomas (DPS), these symptoms occur in less than 10%. Endoscopic retrograde cholangio-pancreatography (ERCP), magnetic resonance or computed tomography can aid in diagnosis, which is only confirmed by psammoma bodies on histologic studies, a rare finding in pancreatic tumors (4) . Surgical resection (duodenopancreatectomy) is the treatment of choice. The authors present a case of DPS with cholestasis, endoscopic treated with excellent results and follow-up for 7 years.44-year-old woman with biliary colic for 1 month and evolving with 1-week jaundice. Bilirubin =14 mg/dL (conjugated =12 mg/ dL), AST, ALT and GGT increased and chromogranin A =6 U/l. ERCP revealed choledochal dilatation, presence of spontaneous choledocoduodenal fistula and a papillary tumor. A 10F plastic prosthesis was inserted through the fistulous orifice and biopsy was performed. The pathology report revealed neuroendocrine tumor. Magnetic Resonance Imaging unveiled a tumor on duodenal papilla (2.0 cm) and three peri duodenal lymph nodes (LN). PET/CT (FDG) revealed hypermetabolic area on duodenal papilla without LN involvement (FIGURE 1). Endosonography