title: Undifferentiated carcinoma of the ampulla of Vater.
abstract: 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.
authors.affiliation: Department of Gastrointestinal Surgery, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Misuzu
authors.initials: M
authors.lastname: Yamada
authors.affiliation: Department of Gastrointestinal Surgery, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, 259-1193, Japan. furufuru@is.icc.u-tokai.ac.jp.
authors.forename: Daisuke
authors.initials: D
authors.lastname: Furukawa
authors.affiliation: Department of Gastrointestinal Surgery, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Naoki
authors.initials: N
authors.lastname: Yazawa
authors.affiliation: Department of Gastrointestinal Surgery, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Hideki
authors.initials: H
authors.lastname: Izumi
authors.affiliation: Department of Gastrointestinal Surgery, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Yoshihito
authors.initials: Y
authors.lastname: Masuoka
authors.affiliation: Department of Gastrointestinal Surgery, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Taro
authors.initials: T
authors.lastname: Mashiko
authors.affiliation: Department of Internal Medicine, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Kawaguchi
authors.affiliation: Department of Internal Medicine, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Masami
authors.initials: M
authors.lastname: Ogawa
authors.affiliation: Department of Internal Medicine, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Yohei
authors.initials: Y
authors.lastname: Kawashima
authors.affiliation: Department of Internal Medicine, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Mine
authors.affiliation: Department of Pathology, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Hirabayashi
authors.affiliation: Department of Gastrointestinal Surgery, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa, 259-1193, Japan.
authors.forename: Toshio
authors.initials: T
authors.lastname: Nakagohri
keywords: Ampulla of Vater; Cisplatin; Gemcitabine; Pancreaticoduodenectomy; Undifferentiated carcinoma