A case of small cell carcinoma of the ampulla of vater

Abstract

We report a case of small cell carcinoma (endocrine cell carcinoma) of the ampulla of Vater. A 54–year–old man was admitted to our hospital due to obstructive jaundice and loss of appetite. Endoscopic study identified a protruded tumor at the ampulla of the Vater. Histological examination showed round shaped atypical cells and positive staining for CD–56 and NSE immunohistochemically. From these fidings, this tumor was preoperatively diagnosed as small cell carcinoma of the ampulla of Vater. We performed subtotal stomach preserving pancreaticodeuodenectomy with regional lymph nodes dissection. Thereafter, 4 cycles of adjuvant chemotherapy with CBDCA and CPT–11 were also given. Nevertheless, the celiac lymph node metastasis was detected within half a year postoperatively. We conducted radiation with simultaneous chemotherapy with CDDP and VP–16. Responding to those therapies, matastatic lymph nodes were markedly decreased in size. However, the patient died of multiple organ failure following multiple bone metastases 247 days after the operation. Although only 35 cases of small cell carcinoma of the ampulla of Vater have been reported in Japan, prognoses of these cases are very poor. It is important to explore more effective multidisciplinary treatments in order to improve the prognosis of patient with extrapulmonary small cell carcinoma.