婴儿左臀部混杂性神经纤维瘤-神经束膜瘤1例

A Case of Mixed Neurofibromatosis-Perineuriomas of the Left Hip in an Infant

目的探讨混杂性神经纤维瘤-神经束膜瘤的病理和影像特点,以提高对该病的认识。方法回顾性分析1例经病理证实的左臀部混杂性神经纤维瘤-神经束膜瘤患儿的临床资料,并结合相关文献进行分析。结果患者临床表现为左臀部无痛性囊实性肿物,超声表现为实性不均质回声,内可及点线状血流信号,MRI表现为团块状T_(1)WI低信号、T_(2)WI稍低信号,压脂呈不均匀高信号,术后病理证实为混杂性神经纤维瘤-神经束膜瘤,术后恢复良好。结论混杂性神经纤维瘤-神经束膜瘤临床罕见,需进行组织病理学明确诊断,治疗以手术局部切除为主,预后良好。

Objective To investigate the pathological and imaging features of hybrid neurofibroma/perineurioma in order to improve the understanding of the disease.Methods Retrospective analysis of the clinical data of an infant with pathologically confirmed left gluteal hybrid neurofibroma/perineurioma in the context of the relevant literature.Results The patient presented clinically with a painless cystic solid mass on the left buttock,ultrasound showed solid inhomogeneous echogenicity with dotted line blood flow signal,MRI showed a mass T_(1)WI low signal,T_(2)WI slightly low signal,and uneven high signal in compression lipids postoperative pathology confirmed a hybrid neurofibroma/perineurioma with good postoperative recovery.Conclusion Hybrid neurofibroma/perineurioma are rare clinically and require histopathology for a clear diagnosis,treatment is based on surgical local excision with a good prognosis.