Abstract
We report an extremely rare case of small cell carcinoma of the ampulla of Vater. A 72-year-old Japanese female, who had been followed due to chronic hepatitis C, was referred to our hospital with a diagnosis of carcinoma of the ampullary region according to the pathological findings of endoscopic biopsy specimens. An operation of pancreatoduodenectomy reconstructed by a modified Child's method was performed with lymph node dissection. The detailed histological and immunohistochemical examination revealed that the tumor was identical to extrapulmonary small cell carcinoma combined with tubular epithelial differentiation and that neuroendocrine differentiation was clarified by chromogranin A immuno-staining. This tumor was smallest ever reported in the Japanese literature, and involved no lymph node. It is well known that this disease has an extremely poor prognosis. Although the present tumor was very small and had no metastatic lymph node, she died of progressive hepatic metastases one year after the surgery. A standard chemotherapy for gastrointestinal small cell carcinoma should be established.
