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

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2 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)537-542
Number of pages6
JournalWorld journal of gastroenterology
Volume24
Issue number4
DOIs
Publication statusPublished - 2018 Jan 28

Keywords

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

ASJC Scopus subject areas

  • Gastroenterology

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  • Cite this

    Lee, J. M., Lee, J. M., Hyun, J. J., Choi, H. S., Kim, E. S., Keum, B., Jeen, Y. T., Chun, H. J., Lee, H. S., Kim, C. D., Kim, D. S., & Kim, J. Y. (2018). Intraductal papillary bile duct adenocarcinoma and gastrointestinal stromal tumor in a case of neurofibromatosis type 1. World journal of gastroenterology, 24(4), 537-542. https://doi.org/10.3748/wjg.v24.i4.537