title: Small bowel adenocarcinoma arising in a patient with hereditary hemorrhagic telangiectasia: A case report.
abstract: Patients with hereditary hemorrhagic telangiectasia (HHT) are reportedly at a lower overall risk of malignancies, and small bowel adenocarcinoma (SBA) arising in a HHT patient is extremely rare. In this study, the case of a 37-year-old female with HHT who developed a poorly differentiated jejunal adenocarcinoma five years after ileocecal resection for multiple colonic adenomas is presented. The patient underwent curative resection of the cancer invading the ileum and the mesentery of the transverse colon, but had to overcome critical complications perioperatively, stemming from HHT-associated peripheral capillary dilatation and arteriovenous malformation, including nosebleeds and possible infusion-induced air embolism through pulmonary shunts. The patient subsequently received adjuvant chemotherapy including capecitabine and oxaliplatin for 6 months, and currently remains alive without any evidence of recurrence 12 months after the second surgery. This patient with SBA was an instructive case demonstrating the necessity of careful attention during major surgery in HHT.
authors.affiliation: Department of Surgical Oncology, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Yuichiro
authors.initials: Y
authors.lastname: Yoshioka
authors.affiliation: Department of Surgical Oncology, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Nozawa
authors.affiliation: Department of Surgical Oncology, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Junichiro
authors.initials: J
authors.lastname: Tanaka
authors.affiliation: Department of Surgical Oncology, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Nishikawa
authors.affiliation: Department of Surgical Oncology, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Tanaka
authors.affiliation: Department of Surgical Oncology, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Tomomichi
authors.initials: T
authors.lastname: Kiyomatsu
authors.affiliation: Department of Surgical Oncology, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Kazushige
authors.initials: K
authors.lastname: Kawai
authors.affiliation: Department of Surgical Oncology, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Keisuke
authors.initials: K
authors.lastname: Hata
authors.affiliation: Department of Surgical Oncology, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Shinsuke
authors.initials: S
authors.lastname: Kazama
authors.affiliation: Department of Surgical Oncology, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Hironori
authors.initials: H
authors.lastname: Yamaguchi
authors.affiliation: Department of Surgical Oncology, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Soichiro
authors.initials: S
authors.lastname: Ishihara
authors.affiliation: Department of Surgical Oncology, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Eiji
authors.initials: E
authors.lastname: Sunami
authors.affiliation: Department of Surgical Oncology, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Joji
authors.initials: J
authors.lastname: Kitayama
authors.affiliation: Department of Surgical Oncology, University of Tokyo, Tokyo 113-8655, Japan.
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Watanabe
keywords: chemotherapy; complications; hereditary hemorrhagic telangiectasia; small bowel adenocarcinoma; surgical management