© Copyright 2008 by the Society for Neuro-Oncology
Received September 19, 2007
Accepted January 4, 2008
Assessment of benign tumor burden by whole-body MRI in patients with neurofibromatosis 1
Victor-F. Mautner 1,
Florence A. Asuagbor, 2,
Eva Dombi 3,
Carsten Fünsterer 4,
Lan Kluwe 5,
Ralf Wenzel 4,
Brigitte C. Widemann 3,
Jan M. Friedman 6*
1 Department of Maxillofacial Surgery, University Hospital Eppendorf, Hamburg, Germany
2 Department of Maxillofacial Surgery, University Hospital Eppendorf, Hamburg, Germany; MRI Institute, Hamburg Othmarschen, Germany
3 Pediatric Oncology Branch, National Cancer Institute, Bethesda, MD, USA
4 MRI Institute, Hamburg Othmarschen, Germany
5 Laboratory for Tumor Biology and Developmental Disorders, University Hospital Eppendorf, Hamburg, Germany
6 Department of Medical Genetics, University of British Columbia, Vancouver, Canada
* To whom correspondence should be addressed. E-mail: frid{at}interchange.ubc.ca.
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Abstract |
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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 neurofibromas. 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 neurofibromas, 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.
Key Words:
malignant peripheral nerve sheath tumor, MRI, neurofibromas, neurofibromatosis 1
