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CASE REPORT article

Front. Oncol.
Sec. Surgical Oncology
Volume 14 - 2024 | doi: 10.3389/fonc.2024.1336539

Duodenal ampulla neuroendocrine tumor and gastrointestinal stromal tumors in a case of neurofibromatosis type 1: a case report

Provisionally accepted
Tingting Zhang Tingting Zhang 1Peng Zheng Peng Zheng 1*Jiaqi Chen Jiaqi Chen 1*Bo Meng Bo Meng 1*Dapeng Qiu Dapeng Qiu 1*Xianzhou Zhang Xianzhou Zhang 1Feng Han Feng Han 1*Hao Zhuang Hao Zhuang 2*Lu Zheng Lu Zheng 3*
  • 1 Department of Hepatobiliopancreatic Surgery, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital, Zhengzhou, China
  • 2 Department of Hepatobiliopancreatic Surgery, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital, Zhengzhou 450008, Zhengzhou, China
  • 3 Department of Medical Oncology, Affiliated Cancer Hospital of Zhengzhou University, Zhengzhou, Henan Province, China

The final, formatted version of the article will be published soon.

    Neurofibromatosis type 1 (NF-1) is commonly associated with a variety of rare tumors. However, no case of multiple gastric gastrointestinal stromal tumors (GISTs) or duodenal ampulla neuroendocrine tumors (NETs) with multiple liver metastases in an NF-1 patient has yet been reported. We here describe a case of a 55-year-old female patient with NF-1 whose serum proGRP levels were elevated. Gastrointestinal endoscopy and biopsy showed duodenal papilla occupying space, and the pathological diagnosis turned out to be neuroendocrine tumors (NETs). During surgical exploration, multiple tumors were found on the serosal surface of the stomach and numerous miliary metastases in the liver. Following histopathological examination, it was determined that the liver metastases were neurofibromatosis type 1 (NF-1) and the tumors in the gastric wall were gastrointestinal stromal tumors (GISTs).The patient benefited from targeted therapy, and had an uneventful hospital stay. In this case, we emphasize treating patients of neurofibromatosis type 1 who exhibit abdominal symptoms with a high degree of clinical suspicion and performing thorough evaluations to rule out multiple tumors.

    Keywords: case report, neurofibromatosis type 1, Gastrointestinal Stromal Tumors, Neuroendocrine Tumors, surufatinib

    Received: 10 Nov 2023; Accepted: 29 Aug 2024.

    Copyright: © 2024 Zhang, Zheng, Chen, Meng, Qiu, Zhang, Han, Zhuang and Zheng. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence:
    Peng Zheng, Department of Hepatobiliopancreatic Surgery, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital, Zhengzhou, China
    Jiaqi Chen, Department of Hepatobiliopancreatic Surgery, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital, Zhengzhou, China
    Bo Meng, Department of Hepatobiliopancreatic Surgery, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital, Zhengzhou, China
    Dapeng Qiu, Department of Hepatobiliopancreatic Surgery, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital, Zhengzhou, China
    Feng Han, Department of Hepatobiliopancreatic Surgery, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital, Zhengzhou, China
    Hao Zhuang, Department of Hepatobiliopancreatic Surgery, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital, Zhengzhou 450008, Zhengzhou, China
    Lu Zheng, Department of Medical Oncology, Affiliated Cancer Hospital of Zhengzhou University, Zhengzhou, 450008, Henan Province, China

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