Small bowel adenocarcinoma arising in a patient with hereditary hemorrhagic telangiectasia: A case report

Yoshioka,Yuichiro; Nozawa,Hiroaki; Tanaka,Junichiro; Nishikawa,Takeshi; Tanaka,Toshiaki; Kiyomatsu,Tomomichi; Kawai,Kazushige; Hata,Keisuke; Kazama,Shinsuke; Yamaguchi,Hironori; Ishihara,Soichiro; Sunami,Eiji; Kitayama,Joji; Watanabe,Toshiaki

doi:10.3892/ol.2016.4173

Small bowel adenocarcinoma arising in a patient with hereditary hemorrhagic telangiectasia: A case report

Authors:
- Yuichiro Yoshioka
- Hiroaki Nozawa
- Junichiro Tanaka
- Takeshi Nishikawa
- Toshiaki Tanaka
- Tomomichi Kiyomatsu
- Kazushige Kawai
- Keisuke Hata
- Shinsuke Kazama
- Hironori Yamaguchi
- Soichiro Ishihara
- Eiji Sunami
- Joji Kitayama
- Toshiaki Watanabe
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Affiliations: Department of Surgical Oncology, University of Tokyo, Tokyo 113‑8655, Japan
Published online on: February 1, 2016 https://doi.org/10.3892/ol.2016.4173
Pages: 2137-2139

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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.

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