Sata Naohiro, Tsukahara Munetoshi, Koizumi Masaru, Yoshizawa Koji, Kurihara Katsumi, Nagai Hideo, Someya Tsutomu, Saito Ken
Department of Surgery, Jichi Medical School, 3311-1 Yakushiji Minami-kawachi Tochigi, Japan.
World J Surg Oncol. 2004 Aug 15;2:28. doi: 10.1186/1477-7819-2-28.
Small-cell neuroendocrine carcinoma in the duodenum is an extremely rare neoplasm with poor prognosis.
A 57-year-old man presented with sudden onset gastrointestinal bleeding and fainting attacks. Duodenoscopy and hypotonic duodenography revealed a 3 x 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.
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.
十二指肠小细胞神经内分泌癌是一种极其罕见的肿瘤，预后较差。
一名57岁男性出现突发胃肠道出血和晕厥发作。十二指肠镜检查和低张十二指肠造影显示，在十二指肠第二部距 Vater 壶腹相对处有一个3×3 cm的突出肿瘤伴溃疡形成。对肿瘤进行了局部切除，随后用5-氟尿嘧啶和亚叶酸进行辅助化疗。通过免疫组织化学和电子显微镜检查肿瘤表明其本质为神经内分泌性，表达突触素和AE1/AE3，并含有致密核心颗粒。患者无复发迹象，术后无病生存期已超过48个月。
十二指肠小细胞神经内分泌癌的大多数病例显示疾病进展迅速，即使进行根治性手术加或不加化疗也无法避免死亡。我们报告了一种罕见的小细胞神经内分泌癌亚型。如果病变远离 Vater 壶腹，这种亚型似乎预后要好得多，且可能适合局部切除。
