[Cephalic duodenopancreatectomy for endocrine tumor of the ampulla of Vater and of the minor papilla].

OBJECTIVES

Endocrine tumors of the ampulla of Vater and minor papilla are rare. This study describes the mode of presentation and evaluates the correlation between pathological features and prognosis.

PATIENTS: Between 1982 and 1998, 6 patients (3 M, 3 F, 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.