Malignant peripheral nerve sheath tumours in neurofibromatosis type 1

TY - JOUR

T1 - Malignant peripheral nerve sheath tumours in neurofibromatosis type 1

T2 - MRI supports the diagnosis of malignant plexiform neurofibroma

AU - Mautner, V F

AU - Friedrich, R E

AU - von Deimling, A

AU - Hagel, C

AU - Korf, B

AU - Knöfel, M T

AU - Wenzel, R

AU - Fünsterer, C

PY - 2003/9/1

Y1 - 2003/9/1

N2 - 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 tumours, of whom seven had atypical pain, tumour growth or neurological deficits indicative of malignancy; the other 43 were asymptomatic. On MRI all seven symptomatic patients had inhomogeneous lesions, due to necrosis and haemorrhage and patchy contrast enhancement. In one patient, the multiplicity of confluent tumours with inhomogeneous areas in addition to central lesions did not allow exclusion of malignancy. Only three of the 43 asymptomatic patients had comparable changes; the other 40 patients had tumours being of relatively homogeneous structure on T1- and T2-weighted images before and after contrast enhancement. All three asymptomatic patients with inhomogeneous lesions were shown to have MPNST.

AB - 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 tumours, of whom seven had atypical pain, tumour growth or neurological deficits indicative of malignancy; the other 43 were asymptomatic. On MRI all seven symptomatic patients had inhomogeneous lesions, due to necrosis and haemorrhage and patchy contrast enhancement. In one patient, the multiplicity of confluent tumours with inhomogeneous areas in addition to central lesions did not allow exclusion of malignancy. Only three of the 43 asymptomatic patients had comparable changes; the other 40 patients had tumours being of relatively homogeneous structure on T1- and T2-weighted images before and after contrast enhancement. All three asymptomatic patients with inhomogeneous lesions were shown to have MPNST.

KW - Adolescent

KW - Adult

KW - Diagnosis, Differential

KW - Female

KW - Hemorrhage

KW - Humans

KW - Magnetic Resonance Imaging

KW - Male

KW - Middle Aged

KW - Necrosis

KW - Nerve Sheath Neoplasms

KW - Neurofibromatosis 1

KW - Pain

KW - Sensitivity and Specificity

U2 - 10.1007/s00234-003-0964-6

DO - 10.1007/s00234-003-0964-6

M3 - SCORING: Journal article

C2 - 12898075

VL - 45

SP - 618

EP - 625

JO - NEURORADIOLOGY

JF - NEURORADIOLOGY

SN - 0028-3940

IS - 9

ER -