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Journal of Gastroenterology

, Volume 31, Issue 2, pp 263–267 | Cite as

Diffuse ganglioneuromatosis with plexiform neurofibromas limited to the gastrointestinal tract involving a large segment of small intestine

  • Keiji Hirata
  • Kohtaro Kitahara
  • Yasuhiro Momosaka
  • Hiromi Kouho
  • Naoki Nagata
  • Hiroshi Hashimoto
  • Hideaki Itoh
Case Report

Abstract

A rare case of diffuse ganglioneuromatosis with plexiform neurofibromas limited to the gastrointestinal tract in a 20-year-old man is reported. The patient had no cafe-au-lait spots or any apparent tumorous lesions affecting other organs. A fan-shaped resection of the mesentery, including 200 cm of the ileum, together with ileocecal resection was done, and the diagnosis was histologically confirmed. The relationship between this benign tumor and other neurogenic disorders is discussed, along with a review of the literature. Long-term follow up of this patient is required because of the possible development of von Recklinghausen's disease or multiple endocrine neoplasia type IIb.

Key words

diffuse ganglioneuromatosis plexiform neurofibroma von Recklinghausen's disease multiple endocrine neoplasia type IIb 

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Copyright information

© Springer-Verlag 1996

Authors and Affiliations

  • Keiji Hirata
    • 1
  • Kohtaro Kitahara
    • 1
  • Yasuhiro Momosaka
    • 2
  • Hiromi Kouho
    • 3
  • Naoki Nagata
    • 1
  • Hiroshi Hashimoto
    • 3
  • Hideaki Itoh
    • 1
  1. 1.First Department of SurgeryUniversity of Occupational and Environmental HealthKitakyushuJapan
  2. 2.Department of Internal MedicineUniversity of Occupational and Environmental HealthKitakyushuJapan
  3. 3.Department of PathologyUniversity of Occupational and Environmental HealthKitakyushuJapan

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