Yamada Misuzu, Furukawa Daisuke, Yazawa Naoki, Izumi Hideki, Masuoka Yoshihito, Mashiko Taro, Kawaguchi Yoshiaki, Ogawa Masami, Kawashima Yohei, Mine Tetsuya, Hirabayashi Kenichi, Nakagohri Toshio
Department of Gastrointestinal Surgery, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, 259-1193, Japan.
Department of Internal Medicine, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, 259-1193, Japan.
Surg Case Rep. 2017 Dec;3(1):9. doi: 10.1186/s40792-016-0284-9. Epub 2017 Jan 6.
Undifferentiated carcinoma of the ampulla of Vater is a rare disease with unclear and clinical characteristics and prognosis. Here, we report the case of a 61-year-old man with undifferentiated carcinoma of the ampulla of Vater. He presented to our hospital with an increase in hepatobiliary system enzymes that was detected during a health check-up. Imaging and endoscopy demonstrated a tumor with ulcer in the ampulla of Vater, which was diagnosed as a carcinoma by biopsy. No distant metastasis was observed. Subtotal stomach-preserving pancreaticoduodenectomy was performed. Undifferentiated carcinoma was confirmed based on the presence of small round atypical cells with the formation of a solid alveolar lesion on histopathological examination and immunohistochemical staining that was positive for CAM 5.2 but negative for chromogranin A and synaptophysin. The tumor infiltrated the duodenum, but not the pancreas; no lymph node metastasis was observed. However, liver metastases were detected 2 months postoperatively. Chemotherapy was performed, and the tumor size temporality decreased; however, it grew in size again, and the patients subsequently died of the primary disease 15 months postoperatively. Undifferentiated carcinoma of the ampulla of Vater is a very rare histological type. More number of cases is necessary to clarify optimal treatment.
壶腹未分化癌是一种罕见疾病，其临床表现和预后尚不清楚。在此，我们报告一例61岁壶腹未分化癌男性患者。他在健康体检时被检测出肝胆系统酶升高，随后到我院就诊。影像学和内镜检查显示壶腹部有一伴有溃疡的肿瘤，活检诊断为癌，未发现远处转移。遂行保留胃的胰十二指肠次全切除术。组织病理学检查发现小圆形非典型细胞，形成实性肺泡样病变，免疫组化染色显示CAM 5.2阳性，但嗜铬粒蛋白A和突触素阴性，确诊为未分化癌。肿瘤侵犯十二指肠，但未侵犯胰腺，未观察到淋巴结转移。然而，术后2个月发现肝转移。进行了化疗，肿瘤大小暂时缩小，但随后又增大，患者术后15个月死于原发性疾病。壶腹未分化癌是一种非常罕见的组织学类型。需要更多病例来明确最佳治疗方案。
