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 journalArticle

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
Externally publishedYes

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Neurofibromatosis 1
Neurilemmoma
Tumor Burden
Plexiform Neurofibroma
Neurofibroma
Body Burden
Whole Body Imaging
Neurofibromatoses
Fibroma
Physical Examination
Early Diagnosis

Keywords

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

ASJC Scopus subject areas

  • Cancer Research
  • Oncology
  • Clinical Neurology

Cite this

Mautner, V. F., Asuagbor, F. A., Dombi, E., Fünsterer, C., Kluwe, L., Wenzel, R., ... Friedman, J. M. (2008). Assessment of benign tumor burden by whole-body MRI in patients with neurofibromatosis 1. Neuro-Oncology, 10(4), 593-598. https://doi.org/10.1215/15228517-2008-011

Assessment of benign tumor burden by whole-body MRI in patients with neurofibromatosis 1. / Mautner, Victor F.; Asuagbor, Florence A.; Dombi, Eva; Fünsterer, Carsten; Kluwe, Lan; Wenzel, Ralf; Widemann, Brigitte C.; Friedman, Jan M.

In: Neuro-Oncology, Vol. 10, No. 4, 08.2008, p. 593-598.

Research output: Contribution to journalArticle

Mautner, VF, Asuagbor, FA, Dombi, E, Fünsterer, C, Kluwe, L, Wenzel, R, Widemann, BC & Friedman, JM 2008, 'Assessment of benign tumor burden by whole-body MRI in patients with neurofibromatosis 1', Neuro-Oncology, vol. 10, no. 4, pp. 593-598. https://doi.org/10.1215/15228517-2008-011
Mautner VF, Asuagbor FA, Dombi E, Fünsterer C, Kluwe L, Wenzel R et al. Assessment of benign tumor burden by whole-body MRI in patients with neurofibromatosis 1. Neuro-Oncology. 2008 Aug;10(4):593-598. https://doi.org/10.1215/15228517-2008-011
Mautner, Victor F. ; Asuagbor, Florence A. ; Dombi, Eva ; Fünsterer, Carsten ; Kluwe, Lan ; Wenzel, Ralf ; Widemann, Brigitte C. ; Friedman, Jan M. / Assessment of benign tumor burden by whole-body MRI in patients with neurofibromatosis 1. In: Neuro-Oncology. 2008 ; Vol. 10, No. 4. pp. 593-598.
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AB - 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.

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