Case Report
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jan 28, 2018; 24(4): 537-542
Published online Jan 28, 2018. doi: 10.3748/wjg.v24.i4.537
Intraductal papillary bile duct adenocarcinoma and gastrointestinal stromal tumor in a case of neurofibromatosis type 1
Jung Min Lee, Jae Min Lee, Jong Jin Hyun, Hyuk Soon Choi, Eun Sun Kim, Bora Keum, Yoon Tae Jeen, Hoon Jai Chun, Hong Sik Lee, Chang Duck Kim, Dong Sik Kim, Joo Young Kim
Jung Min Lee, Jae Min Lee, Jong Jin Hyun, Hyuk Soon Choi, Eun Sun Kim, Bora Keum, Yoon Tae Jeen, Hoon Jai Chun, Hong Sik Lee, Chang Duck Kim, Division of Gastroenterology and Hepatology, Department of Internal Medicine, Institute of Gastrointestinal Medical Instrument Research, Korea University College of Medicine, Seoul 02841, South Korea
Jae Min Lee, Division of Gastroenterology and Hepatology, Department of Internal Medicine, Korea University Anam Hospital, Seoul 02841, South Korea
Dong Sik Kim, Division of HBP Surgery and Liver Transplantation, Department of Surgery, Korea University College of Medicine, Seoul 02841, South Korea
Joo Young Kim, Department of Pathology, Korea University College of Medicine, Seoul 02841, South Korea
Author contributions: Lee JM and Kim DS performed the procedure and proposed the study; Lee JM wrote the draft; Hyun JJ, Choi HS, Kim JY and Kim ES performed the literature review; Keum B, Jeen YT, Chun HJ, Lee HS and Kim CD reviewed the draft; all authors read and approved the final manuscript.
Supported by a grant of the Korea Health Technology R&D Project through the Korea Health Industry Development Institute (KHIDI), funded by the Ministry of Health & Welfare, South Korea, No. HI14C3477 and by a grant from Korea University.
Informed consent statement: Written informed consent was obtained from the patient for publication of this case report and any accompanying images.
Conflict-of-interest statement: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Jae Min Lee, MD, PhD, Assistant Professor, Division of Gastroenterology and Hepatology, Department of Internal Medicine, Korea University Anam Hospital, Inchon-ro 73, Seongbuk-gu, Seoul 02841, South Korea. jmlee1202@gmail.com
Telephone: +82-2-9206555 Fax: +82-2-9531943
Received: November 9, 2017
Peer-review started: November 9, 2017
First decision: December 6, 2017
Revised: December 13, 2017
Accepted: December 20, 2017
Article in press: December 20, 2017
Published online: January 28, 2018
Processing time: 77 Days and 19.3 Hours
Abstract

We report our experience with a synchronous case of gastrointestinal stromal tumor (GIST) and intraductal papillary neoplasm of the bile duct (IPNB) in an elderly woman with neurofibromatosis type 1 (NF-1). A 72-year-old woman presented with a 2-mo history of right upper abdominal pain unrelated to diet and indigestion. Fourteen years earlier, she had been diagnosed with NF-1, which manifested as café au lait spots and multiple nodules on the skin. Computed tomography (CT) revealed a multilocular low-density mass with septation, and mural nodules in the right hepatic lobe, as well as a 1.7-cm-sized well-demarcated enhancing mass in the third portion of the duodenum. The patient subsequently underwent right hepatectomy and duodenal wedge resection. We present here the first report of a case involving a synchronous IPNB and GIST in a patient with NF-1. Our findings demonstrate the possibility of various tumors in NF-1 patients and the importance of diagnosis at an early stage

Keywords: Neurofibromatosis type 1; Intraductal papillary neoplasm of the bile duct; Gastrointestinal stromal tumor; Synchronous

Core tip: We reported the world wide first case of intraductal papillary adenocarcinoma of bile duct in neurofibromatosis type 1 (NF-1) patient. Because patients with NF-1 have a mutation of the NF-1 gene associated with multiple tumors such as neuroma, and gastrointestinal stromal tumor, consideration for multiple tumors in NF-1 patients would be helpful.