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Objective: Benign lesions of the major papilla are rare but raise the problem of their medical care. We studied the efficacy, safety, and histology of the endoscopic ampullectomy., Patients and Methods: Forty-two endoscopic resections of the major papilla were undertaken in 23 males and 19 females of a mean age of 63. Five patients (12%) presented with a familial adenomatous polyposis. The assessment of resectability included preoperative histology, and endoscopic ultrasound (EUS) in 26 patients (62%) always showing intra-mucosal lesion. The resection was performed with a duodenoscope, using a diathermic loop with a pure current section., Results: The resection was realized in one piece for 34 patients, in 2-4 fragments for 8 patients. A plastic pancreatic stent was inserted in 26 patients (62%), a plastic biliary stent in 10 patients (24%). There were no deaths but nine complications (21%): six acute pancreatitis (four patients with a pancreatic stent, contrary to the literature), three delayed gastrointestinal bleeding. The final histological result was fibrosis and inflammatory tissue in 7 patients, low-grade dysplasia in 20 patients, high-grade dysplasia or in situ carcinoma in 10 patients, invasive adenocarcinoma in 1 patient, and somatostatinoma in 2 patients (concordance of 72% with the initial histology). The resection was complete in 39 patients (93%). Three patients had additional surgery because of positive margin of resection or bad histology criteria. The median of follow-up in 33 patients with a complete resection was of 15 months, and we did not note any recurrence in 29 patients (88%)., Conclusion: Endoscopic ampullectomy is an efficient treatment for superficial lesions of the papilla, despite a significant but rarely severe morbidity. Preoperative EUS is mandatory, preoperative histology is advisable. Long-term follow-up is necessary.

Somatostatinomas are rare tumors; ampullary somatostatinomas are very rare. We report a case of a small pure somatostatin-producing neuroendocrine tumor of ampulla of Vater in a 54-year-old woman with neither neurofibromatosis nor somatostatinoma syndrome, "incidentally" discovered during an abdominal computed tomography. The patient initially refused other adjunctive exams but after 2 years she was admitted, presenting with itch, night sweats, severe fatigue, and unintentional weight loss. The size of the tumor (1.5 cm) and the other radiologic findings had not changed since the abdominal CT scan 2 years before. The somatostatin, gastrin, glucagons, serotonin, vasoactive intestinal peptide, dopamine, norepinephrine, epinephrine, and calcitonin plasma levels were normal. ERCP-obtained biopsies revealed a neuroendocrine tumor with psammoma bodies; immunohistochemical profile was positive for chromogranin and somatostatin. The patient underwent surgery; intraoperative histologic examination of lymph nodes sampling of perihepatic and periduodenal lymph nodes was negative for metastasis. We performed, therefore, a transduodenal ampullectomy. The patient continues to do well at 3 years' follow-up with no evidence of local or distance recurrence of disease.

We report a case of somatostatinoma of the papilla of Vater with multiple gastrointestinal stromal tumors in a patient with von Recklinghausen's disease (VRD). A 64-year-old woman who had had recurrent attacks of acute pancreatitis and cholangitis was found, on gastroduodenal endoscopy, to have a tumor of the papilla of Vater and multiple submucosal tumors of the stomach and duodenum. Numerous submucosal tumors were observed in the stomach, duodenum, and jejunum, and total excision of the papilla of Vater and resection of the duodenal and jejunal submucosal tumors was performed. The tumor of the papilla of Vater showed the histologic appearance of a dense proliferation of tumor cells in acinar form, from the duodenal mucosa to the muscle layer, and psammoma bodies were revealed within the tumor. Immunohistologically, the tumor cells were intensely positive for somatostatin. The submucosal tumors of the duodenum and jejunum were negative for smooth muscle actin, s-100, and neuron-specific enolase (NSE), and positive for CD34 and c-kit, and they were diagnosed as gastrointestinal stromal tumors (GISTs) according to the strict definition. The only 25 cases of papilla of Vater somatostatinoma associated with VRD to have been reported in the English-language literature since 1982 are reviewed, as well as our own case.