儿童胸腺淋巴上皮瘤样癌的临床及影像学表现

Clinical and Radiological Characteristics of Thymic Lymphoepithelioma-Like Carcinoma in Children

目的:探讨儿童胸腺淋巴上皮瘤样癌(LELC)的临床表现及影像学特点。方法:回顾性分析经手术病理证实的4例胸腺LELC患儿的临床、影像及病理学资料,4例均行CT平扫及增强检查,3例行MRI平扫及增强检查。结果:4例均为男性,年龄8岁8个月~13岁3个月。3例以胸痛为首发症状,1例以双膝关节炎为首发症状。4例均合并EB病毒感染。CT检查显示4例为前纵隔肿块,均合并囊变坏死区,增强后实性部分呈轻-中度强化。3例周围见瘤周增粗血管影。MRI检查示3例肿块实性部分呈稍长T1稍长T2信号影,其中2例瘤体内见多结节影,增强后呈“葡萄串”样改变。3例侵犯邻近大血管。3例患者存在远处转移,其中1例患者术后1年内死亡。结论:儿童胸腺LELC的临床及影像学表现具有一定的特征性,认识这些特征有助于提高其诊断准确率。

Purpose:To investigate the clinical presentations and imaging features of thymic lymphoepithelioma-like carcinoma(LELC)in children.Methods:The clinical,imaging,and pathological data of four children diagnosed with thymic LELC through surgical intervention were retrospectively reviewed.All patients underwent both plain and contrast-enhanced CT scans,and three also had plain and contrast-enhanced MRI.Results:All four cases were male,aged between 8 years and 8 months to 13 years and 3 months.Three cases presented with chest pain as the initial symptom,while one case presented with arthritis of both knees.All four cases were associated with Epstein-Barr virus(EBV)infection.CT scans revealed anterior mediastinal masses in all four cases,each with areas of cystic degeneration and necrosis.Post-contrast scans showed mild to moderate enhancement of the solid components.Peritumoral vascular thickening was observed in three cases.MRI scans revealed that the solid components of the masses in three cases exhibited slightly prolonged T1 and T2 signals.In two of these cases,multiple nodules were observed within the masses.Post-contrast scans demonstrated a"grape cluster"appearance.Invasion of adjacent major blood vessels was identified in all three cases.Distant metastasIs was observed in three patients,with one patient dying within one year post-surgery.Conclusion:The clinical and imaging features of pediatric thymic LELC are distinctive and recognizing these charateristics can improve diagnostic accuracy.