2003
DOI: 10.1007/s00234-003-0964-6
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Malignant peripheral nerve sheath tumours in neurofibromatosis type 1: MRI supports the diagnosis of malignant plexiform neurofibroma

Abstract: Plexiform neurofibroma (PNF) is a typical feature of neurofibromatosis 1 (NF1). About 10% of patients with NF1 develop malignant peripheral nerve-sheath tumours (MPNST), usually arising from PNF, and this is the major cause of poor survival. A better prognosis can be achieved if the tumours are diagnosed at an early stage. Our objective was to establish MRI criteria for MPNST and to test their usefulness in detecting early malignant change in PNF. MRI was performed on 50 patients with NF1 and nerve-sheath tumo… Show more

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Cited by 99 publications
(47 citation statements)
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“…MPNSTs classically lack the target sign that is commonly seen in benign neurofibromas. Findings on MRI that are suggestive of malignancy include: increase in the size of previously stable neurofibromas [38], large size [38], inhomogeneity [35], ill-defined margins, invasion of fat planes [33], and perilesional edema [33]. Accurate grading of MPNSTs by MRI criteria is not possible [38].…”
Section: Radiology Of Mpnstmentioning
confidence: 99%
“…MPNSTs classically lack the target sign that is commonly seen in benign neurofibromas. Findings on MRI that are suggestive of malignancy include: increase in the size of previously stable neurofibromas [38], large size [38], inhomogeneity [35], ill-defined margins, invasion of fat planes [33], and perilesional edema [33]. Accurate grading of MPNSTs by MRI criteria is not possible [38].…”
Section: Radiology Of Mpnstmentioning
confidence: 99%
“…Further, MRI has been suggested as a potentially useful tool in detecting progression to malignancy in NF1 neurofibromas. 44 We are currently exploring the use of MRI in studying the malignant progression of neurofibromas, using NF1 xenograft models of both MPNST and plexiform-like neurofibroma.…”
Section: Discussionmentioning
confidence: 99%
“…Isolated Plexiform Neurofibroma of Arm with Unusual Presentation -A Rare Case Report changes [2,3]. While it is a matter of debate amongst literature, that what is proper time and proper extent of PNF resection.…”
Section: Orthopaedics Sectionmentioning
confidence: 99%