A Case of Ampullary Neuroendocrine Carcinoma

Kojiro Taura, Iwao Ikai, Masayuki Shimoda, Shinya Hamasu, Etsuro Hatano, Hideaki Fujii, Yukari Adachi*, Yasuaki Nakashima* and Yasuyuki Shimahara

Department of Gastroenterological Surgery, Kyoto University Graduate School of Medicine
Laboratory of Anatomic Pathology, Kyoto University Hospital*

A 53-year-old man with epigastralgia was found in endoscopy to have a tumor at the papilla of Vater, necessitating pylorus-preserving pancreaticoduodenectomy. Histological examination showed round, small cells with scanty cytoplasm proliferating in solid and cribriform patterns with glandular fenestrations. Nuclear atypia was prominent and mitoses were frequent. Tumor cells were positive for chromogranin A and CD56, yielding a diagnosis of neuroendocrine carcinoma (small-cell carcinoma). Liver metastases were observed 2 months post operatively. Hepatic arterial infusion chemotherapy with CDDP/CPT-11 was conducted, based on chemotherapy for small-cell carcinoma of the lung. Although this was initially effective and metastasis almost disappeared, the disease progressed very rapidly after relapse and he died 13 months after surgery. Neuroendocrine carcinoma of the gastrointestinal tract is reported to be very aggressive and have a dismal prognosis. Although effective chemotherapy has not been well documented, a chemotherapy regimen based on small-cell lung cancer provides reasonable alternative therapy for this disease.

Key words
neuroendocrine carcinoma, ampullary carcinoma, hepatic arterial infusion chemotherapy

Reprint requests
Kojiro Taura Department of Gastroenterological Surgery, Kyoto University Graduate School of Medicine
54 Kawahara-cho, Shogoin, Sakyo-ku, Kyoto, 606-8507 JAPAN

Accepted
September 28, 2005

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