Case report

Primary small-cell neuroendocrine carcinoma of the duodenum – a case report and review of literature

Naohiro Sata¹ , Munetoshi Tsukahara¹ , Masaru Koizumi¹ , Koji Yoshizawa¹ , Katsumi Kurihara¹ , Hideo Nagai¹ , Tsutomu Someya² and Ken Saito²

¹  Department of Surgery, Jichi Medical School, 3311-1 Yakushiji Minami-kawachi Tochigi, Japan

²  Department of Pathology, Jichi Medical School, 3311-1 Yakushiji Minami-kawachi Tochigi, Japan

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World Journal of Surgical Oncology 2004, 2:28doi:10.1186/1477-7819-2-28

Published:	15 August 2004

Abstract

Background

Small-cell neuroendocrine carcinoma in the duodenum is an extremely rare neoplasm with poor prognosis.

Case presentation

A 57-year-old man presented with sudden onset gastrointestinal bleeding and fainting attacks. Duodenoscopy and hypotonic duodenography revealed a 3 × 3 cm protruding tumor with ulcerations situated opposite the ampulla of Vater in the second part of the duodenum. Local excision of the tumor was performed, followed by adjuvant chemotherapy with 5-fluoro uracil and leucovorin. Examination of the tumor by immunohistochemistry and electron microscopy indicated it to be neuroendocrine in nature, expressing synaptophysin and AE1/AE3, and containing dense core granules. The patient showed no sign of recurrence and has been disease-free for more than 48 months after surgery.

Conclusions

Most cases of small-cell neuroendocrine carcinoma in the duodenum show rapid progression of the disease, and even radical surgery with or without chemotherapy do not prevent death. We report a rare subtype of small-cell neuroendocrine carcinoma. This subtype appears to have a much better prognosis, and may be amenable to local excision, if the lesion is away from the ampulla of Vater.