软组织恶性外周神经鞘瘤的临床及MRI表现

Clinical and MRI Features of Malignant Peripheral Nerve Sheath Tumor in Soft Tissue

目的分析软组织恶性外周神经鞘瘤(MPNST)患者的临床及MRI表现,提高术前对其诊断的准确性。方法回顾性分析14例经病理证实的软组织MPNST患者的临床及MRI表现。结果 14例MPNST患者临床均表现为伴压痛或触痛的局部肿块,其中1例伴有神经纤维瘤病Ⅰ型。MRI多表现为位于肌肉层(6/14)的单发(11/14)肿块,呈类圆形(9/14)或不规则形(5/14),边缘多欠光整(10/14),有不完整包膜(8/14),最大径平均10.3 cm。T1WI、T2WI脂肪抑制边缘以等信号为主,内部可见不规则坏死区,周围软组织可见水肿改变(10/14),增强扫描多呈明显不均匀强化(10/14)。结论软组织MPNST的临床与MRI表现有一定特征,对提高其术前诊断准确性有一定的意义。

Objective To analyze clinical and MRI features of malignant peripheral nerve sheath tumors in soft tissue, and to improve its preoperative diagnostic accuracy. Methods Clinical and MRI features of 14 patients with histologically diagnosed soft tissue MPNST were analyzed retrospectively. Results Clinical features of all cases were localized mass with tenderness, one cases was accompanied with neurofibromatosis I. Most of them were solitary mass located in muscular lay- er, circular（9/14） or irregular shape（5/14）, irregular edge（ 10/14）, incomplete capsule（8/14）, the average maximum diameter was 10.3 era. MR features were mainly isointense relative to muscle on T1 weighted images and T2 weighted FS images,irregular necrosis zone in intratumoral and perilesion edema like zone （10/14） were seen with inhomogeneous en- hancement （10/14）. Conclusion Clinical and MRI manifestations of soft tissue MPNST have certain characteristics, which have certain significance to improve the preoperative diagnostic accuracy.