Multiple gastrointestinal stromal tumors of the small intestine in a patient with neurofibromatosis type 1: a case report

DOI Open Access
  • Mori Yoshiko
    Departments of Gastroenterological Surgery, Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences, Okayama, Japan.
  • Shigeyasu Kunitoshi
    Departments of Gastroenterological Surgery, Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences, Okayama, Japan.
  • Yoshioka Takahiro
    Departments of Gastroenterological Surgery, Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences, Okayama, Japan.
  • Nagasaka Takeshi
    Department of Clinical Oncology, Kawasaki Medical School, Kurashiki City, Japan.
  • Haraga Junko
    Obstetrics and Gynecology, Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences, Okayama, Japan.
  • Kagawa Shunsuke
    Departments of Gastroenterological Surgery, Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences, Okayama, Japan.
  • Teraishi Fuminori
    Departments of Gastroenterological Surgery, Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences, Okayama, Japan.
  • Toyooka Shinichi
    Department of General Thoracic Surgery and Breast and Endocrinological Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
  • Hirasawa Akira
    Department of Clinical genomic Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
  • Fujiwara Toshiyoshi
    Departments of Gastroenterological Surgery, Okayama University Graduate School of Medicine Dentistry and Pharmaceutical Sciences, Okayama, Japan.

Bibliographic Information

Other Title
  • 多発小腸GISTに対し手術を施行した神経線維腫症1型の1例

Abstract

Neurofibromatosis type 1 (NF1) is a complex autosomal dominant disorder caused by germline variants in the NF1 tumor suppressor gene characterized by multiple caféau lait spots and cutaneous neurofibromas. Therefore, NF1 predisposes patients to benign and malignant tumor development. We report a 54-year-old NF1 male with multiple gastrointestinal stromal tumors (GIST) in the small intestine. We resected a part of the small intestine with larger tumors, but left the part with small tumors to avoid short bowel syndrome. Histological examination revealed spindle cells with eosinophilic cytoplasm. The tumors were positive for KIT on immunopathological examination. They were smaller than 3.5 cm and their mitotic activity was less than 5/50 in high-power fields. We left 17 GIST that were smaller than 10 mm, but no progression has been detected to date. (<250 words)

Journal

Related Projects

See more

Keywords

Details 詳細情報について

  • CRID
    1390283659855276544
  • NII Article ID
    130007803646
  • DOI
    10.18976/jsft.19.2_77
  • ISSN
    21896674
    13461052
  • Text Lang
    ja
  • Data Source
    • JaLC
    • CiNii Articles
    • KAKEN
  • Abstract License Flag
    Disallowed

Report a problem

Back to top