Format

Send to

Choose Destination
World J Gastroenterol. 2018 Jan 28;24(4):537-542. doi: 10.3748/wjg.v24.i4.537.

Intraductal papillary bile duct adenocarcinoma and gastrointestinal stromal tumor in a case of neurofibromatosis type 1.

Author information

1
Division of Gastroenterology and Hepatology, Department of Internal Medicine, Institute of Gastrointestinal Medical Instrument Research, Korea University College of Medicine, Seoul 02841, South Korea. jmlee1202@gmail.com.
2
Division of Gastroenterology and Hepatology, Department of Internal Medicine, Institute of Gastrointestinal Medical Instrument Research, Korea University College of Medicine, Seoul 02841, South Korea.
3
Division of HBP Surgery and Liver Transplantation, Department of Surgery, Korea University College of Medicine, Seoul 02841, South Korea.
4
Department of Pathology, Korea University College of Medicine, Seoul 02841, South Korea.

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:

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

PMID:
29398874
PMCID:
PMC5787788
DOI:
10.3748/wjg.v24.i4.537
[Indexed for MEDLINE]
Free PMC Article

Conflict of interest statement

Conflict-of-interest statement: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

Supplemental Content

Full text links

Icon for Baishideng Publishing Group Inc. Icon for PubMed Central
Loading ...
Support Center