A Case of Small-Cell Carcinoma (Endocrine Cell Carcinoma) of the Ampulla of Vater: A Case Report and Literal Review

Takashi Kobayashi, Masanori Teruya, Seiichiro Shimizu*, Akihiko Ito, Kenji Miki, Kaoru Kobayashi and Koji Morita

Departments of Surgery and Pathology*, Showa General Hospital

We report a case of small-cell carcinoma (endocrine cell carcinoma) of the ampulla of Vater. A 73-year-old woman admitted for appetite loss, jaundice, and high fever was found to have an elevated tumor with ulceration in the ampulla of Vater. Histological examination of a biopsied specimen showed poorly differentiated adenocarcinoma. We conducted pancreatoduodenectomy with regional lymph node dissection. Immunohistochemical analysis showed positive staining for NSE, CD-56, synaptophysin, and chromogranin A. The definitive diagnosis was small-cell carcinoma (endocrine cell carcinoma) of the ampulla of Vater. The postoperative course was uneventful, but the patient died from recurrence at the hepatic hilum 10 months after surgery. To the best of our knowledge, this is the 34th case of small-cell carcinoma (endocrine cell carcinoma) of the ampulla of Vater reported in the Japanese literature. The prognosis in surgical treatment of such cases is dismal, mainly due to early liver metastasis. Adjuvant chemotherapy in addition to surgery may thus be necessary.

Key words
small-cell carcinoma, endocrine cell carcinoma, the ampulla of Vater

Reprint requests
Takashi Kobayashi Department of Surgery, Showa General Hospital
2-450 Tenjincho, Kodaira, 187-8510 JAPAN

Accepted
June 22, 2005

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