婴儿左侧颞部低度恶性肌纤维母细胞肉瘤1例报告及文献复习

Left temporal low-grade myofibroblastic sarcoma of infant:A case report and literature review

目的:分析左侧颞部低度恶性肌纤维母细胞肉瘤(LGMS)患儿的临床表现和治疗方法,为其诊断提供依据。方法:收集1例左侧颞部LGMS患儿的临床资料,结合相关文献,分析LGMS的临床特点、诊断和治疗方法。结果:患儿为4月龄男婴,因发现左侧颞部肿物20余天入院。头部CT,左侧颞部皮下占位性病变,CT值为20~40Hu,大小约为2.0cm×2.0cm×2.0cm,病变侵入颅内。头部MRI平扫+增强示左侧颞骨局部见不规则结节影,病变侵入颅内,与周围脑组织边界尚清,大小约为1.9cm×1.6cm×2.0cm,T1WI、T2WI和FLAIR均呈等信号,增强扫描呈不均匀强化。头部CT及MRI检查均显示皮下肿物侵入颅内,提示有恶性肿瘤可能,患儿行左侧颞部皮下颅内外占位切除术将肿物全切,病理诊断为LGMS。患儿术后未接受放、化疗。随访16个月未见复发。结论:LGMS是一种侵袭性肿瘤,无特异性的影像学表现,手术全切是有效的治疗方式,病理学诊断是本病诊断的金标准。LGMS患者术后应定期随访。

Objective:To analyze the clinical features and treatment methods of one infant with low-grade malignant myofibroblastic sarcoma(LGMS)in the left temporal region,and to provide the basis for its diagnosis.Methods:The clinical data of one infant with LGMS in the left temporal region were collected,and the clinical features,diagnosis and treatment of the patient with LGMS were analyzed combined with the relative literatures.Results:A 4-month-old infant was admitted because of left temporal mass for 20 d.The CT image showed a subcutaneous space-occupying lesion in the left temporal region,the CT value was 20-40 HU,and the size was about 2.0 cm × 2.0 cm ×2.0 cm and the lesion invaded the skull.The enhanced MRI images showed an irregular lesion in the left temporal region,the lesion invaded the skull,the border with the surrounding brain tissue was still clear,the size was about 1.9 cm× 1.6 cm× 2.0 cm,and the T1 WI,T2 WI and FLAIR results presented as isointensity in the lesion.The lesion showed inhomogeneous enhancement.The CT and MRI results showed that the subcutaneous tumor invaded the skull,suggesting that there may be malignant tumor.The patient underwent left temporal subcutaneous intracranial and extracranial mass operation to completely resect the lesion,and the pathological diagnosis was LGMS.The patient did not receive radiotherapy or chemotherapy after operation.There was no recurrence after follow-up for 16 months.Conclusion:LGMS is a kind of invasive tumor without specific imaging findings.Total resection is an effective treatment.The pathological diagnosis is the gold standard for the diagnosis of LGMS.The LGMS patients should be given regularly follow-up after operation.