Assessment of benign tumor burden by whole-body MRI in patients with neurofibromatosis 1

Victor F. Mautner, Florence A. Asuagbor, Eva Dombi, Carsten Fünsterer, Lan Kluwe, Ralf Wenzel, Brigitte C. Widemann, Jan M. Friedman

Research output: Contribution to journalArticlepeer-review

Abstract

People with neurofibromatosis 1 (NF1) have multiple benign neurofibromas and a 10% lifetime risk of developing malignant peripheral nerve sheath tumors (MPNSTs). Most MPNSTs develop from benign plexiform neurofibromas, so the burden of benign tumors may be a risk factor for developing MPNST. We studied 13 NF1 patients with MPNSTs and 26 age- and sex-matched controls (NF1 patients who did not have MPNSTs) with detailed clinical examinations and whole-body MRI to characterize their body burden of internal benign neuro- fibromas. Internal plexiform neurofibromas were identified in 22 (56%) of the 39 NF1 patients studied. All six of the NF1 patients with MPNSTs under 30 years of age had neurofibromas visualized on whole-body MRI, compared to only 3 of 11 matched NF1 controls under age 30 (p < 0.05). Both the median number of plexiform neurofibromas (p < 0.05) and the median neurofibroma volume (p < 0.01) on whole-body MRI were significantly greater among MPNST patients younger than 30 years of age than among controls. No significant differences in whole-body MRI findings were observed between NF1 patients with MPNSTs and controls who were 30 years of age or older. Whole-body MRI of NF1 patients allows assessment of the burden of internal neu- rofibromas, most of which are not apparent on physical examination. Whole-body imaging of young NF1 patients may allow those at highest risk for developing MPNST to be identified early in life. Close surveillance of these high-risk patients may permit earlier diagnosis and more effective treatment of MPNSTs that develop.

Original languageEnglish (US)
Pages (from-to)593-598
Number of pages6
JournalNeuro-oncology
Volume10
Issue number4
DOIs
StatePublished - Aug 2008

Keywords

  • MRI
  • Malignant peripheral nerve sheath tumor
  • Neurofibromas
  • Neurofibromatosis 1

ASJC Scopus subject areas

  • Oncology
  • Clinical Neurology
  • Cancer Research

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