Mautner V F, Friedrich R E, von Deimling A, Hagel C, Korf B, Knöfel M T, Wenzel R, Fünsterer C
Department of Neurology, Klinikum Nord Hamburg, Langenhorner Chaussee 560, 22419 Hamburg, Germany.
Neuroradiology. 2003 Sep;45(9):618-25. doi: 10.1007/s00234-003-0964-6. Epub 2003 Jul 24.
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.
丛状神经纤维瘤（PNF）是神经纤维瘤病1型（NF1）的典型特征。约10%的NF1患者会发生恶性外周神经鞘瘤（MPNST），通常由PNF发展而来，这是导致生存率低的主要原因。如果肿瘤能早期诊断，则可获得更好的预后。我们的目的是建立MPNST的MRI标准，并测试其在检测PNF早期恶性变化中的效用。对50例患有NF1和神经鞘瘤的患者进行了MRI检查，其中7例有提示恶性的非典型疼痛、肿瘤生长或神经功能缺损；其他43例无症状。在MRI上，所有7例有症状的患者均有不均匀病变，原因是坏死、出血及斑片状对比增强。1例患者，除中央病变外，融合性肿瘤的多样性及不均匀区域无法排除恶性。43例无症状患者中只有3例有类似变化；其他40例患者的肿瘤在对比增强前后的T1加权和T2加权图像上结构相对均匀。所有3例有不均匀病变的无症状患者均被证实患有MPNST。
