Duodenopancreatectomie cephalique pour tumeur endocrine de l'ampoule de Vater et de la petite caroncule

Objectives - Endocrine tumors of the ampulla of Vater and minor papilla are rare. This study describes the node of presentation and evaluates the correlation between pathological features and prognosis. Patients - Between 1982 and 1998, 6 patients (3M, 3F, mean age: 47.6 years, range: 36-58) for whom a diagnosis of endocrine tumor of the ampulla of Vater or minor papilla was made between 1982 and 1998 after histological examination of an operative specimen of pancreaticoduodenectomy. Results - One patient was detected incidentally, two had a Zollinger-Ellison syndrome, two had pain and one had obstructive jaundice with pain. The tumor was located in the ampulla of Vater in 5 cases and at the minor papilla in 1 case. All patients underwent a pancreaticoduodenectomy, with histological examination showing tumor diameter varying from 5 to 40 mm and positive lymph nodes. Five patients had a well differentiated endocrine tumor and one a poorly differentiated tumor. All patients had positive Grimelius staining. The secretory profile analyzed by immunohistochemistry was heterogeneous. Median duration of follow-up was 51 months (range: 6 months-16 years) with all patients currently still alive. The patient with a poorly differentiated tumor had diffuse liver metastases, the others were disease-free. Conclusion - This study demonstrates the frequency of metastatic spread to adjacent lymph nodes and the inconsistent secretory profiles of these tumors. Pancreaticoduodenectomy may offer long term disease-free survival in well differentiated tumors, and such histology may be useful in advising on prognosis.