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©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
ARTICLE HIGHLIGHTS
Case characteristics
A 72-year-old Asian woman who was diagnosed with neurofibromatosis type 1 (NF-1) presented with right upper abdominal pain.
Clinical diagnosis
The patient had a negative Murphy’s sign, and no evidence of jaundice. There was no palpable mass in right upper quadrant.
Differential diagnosis
Cholecystitis, choledocholithiasis, cholangiocarcinoma, hepatocellular carcinoma
Laboratory diagnosis
Aspartate transaminase, alanine transaminase, and total bilirubin were within normal range. Alkaline phosphatase, γ-glutamyltransferase was increased slightly.Carcinoembryonic antigen is 3.4 ng/mL, however, cancer antigen 19-9 level was elevated to 211.3 IU/mL.
Imaging diagnosis
Computed tomography showed a low-attenuated multilocular mass with septation, and a mural nodule containing a soft tissue-enhancing lesion in the right hepatic lobe. In addition, a 1.7-cm-sized, well-demarcated enhancing mass was observed in the third portion of the duodenum.
Pathological diagnosis
Histologic results confirmed the diagnosis of benign gastrointestinal stromal tumor (GIST) in the third portion of the duodenum. In addition, the resected hepatic mass was intraductal papillary neoplasm of bile duct (IPNB).
Treatment
Surgical resection was curative treatment. Both GIST and IPNB were successfully resected.
Related reports
NF-1 is associated with an increased risk of tumors. Recently, there were related reports: case of a mixed periampullary adenocarcinoma and a somatostatinoma with a gastrointestinal stromal tumor in NF-1 patients, triad of periampullary carcinoid, duodenal gastrointestinal stromal tumor and plexiform neurofibroma at hepatic hilum in NF-1.
Term explanation
IPNB is a variant of bile duct carcinoma that is characterized by intraductal growth. It have better outcomes compared with common cholangiocarcinoma.
Experiences and lessons
Suspicion and early diagnosis are important for asymptomatic patients with NF-1.
