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Mesenteric plexiform neurofibroma associated with Recklinghausen's disease

K Matsuki1, Y Kakitsubata, K Watanabe

  • 1Department of Radiology, Miyazaki Medical College, Japan.

Pediatric Radiology
|March 1, 1997
PubMed

Insights

This study reports a rare case of mesenteric plexiform neurofibroma in a child with Recklinghausen's disease. Characteristic MRI findings, including ring-like structures, aid in diagnosing this condition.

Area of Science:

  • Radiology
  • Pediatric Oncology
  • Gastrointestinal Surgery

Background:

  • Neurofibromatosis type 1 (Recklinghausen's disease) can manifest with various tumors.
  • Plexiform neurofibromas are benign nerve sheath tumors, but can cause complications.
  • Mesenteric involvement is uncommon, presenting diagnostic challenges.

Observation:

  • A 10-year-old boy with Recklinghausen's disease presented with ileal mesenteric masses.
  • Magnetic Resonance Imaging (MRI) revealed multiple ring-like structures within the masses.
  • These structures were observed on T2-weighted and contrast-enhanced T1-weighted sequences.

Findings:

  • The characteristic ring-like appearance on MRI suggests enlarged peripheral nerves with myxoid degeneration.
  • This specific imaging pattern is indicative of a mesenteric plexiform neurofibroma.
  • MRI proved valuable in identifying the nature of the mesenteric masses.

Implications:

  • Early and accurate diagnosis of mesenteric plexiform neurofibroma is crucial for appropriate management.
  • MRI's ability to detect characteristic features aids in differentiating these tumors from other mesenteric masses.
  • This case highlights the importance of considering neurofibromatosis in pediatric patients with unexplained abdominal masses.

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