肾脏畸胎瘤样肾母细胞瘤的病理观察:附1例报告

Teratoid Wilms' tumor:clinicopathologic study of a case and literature review

目的了解畸胎瘤样肾母细胞瘤(teratoid Wilms'tumor)的病理诊断、鉴别诊断和预后。方法分析1例畸胎瘤样肾母细胞瘤患儿的临床资料,病理学检查包括常规病理学、免疫组化,并进行随访。结果光镜下肿瘤由表现为大量异源性组织(角化物质的湖、脂肪、肌肉、软骨和花边样骨样基质)中有岛状的经典肾母细胞瘤的三种成分——胚芽、腺管和基质。免疫组化肿瘤细胞表达肾母细胞瘤蛋白(WT1)阳性。患者手术后26个月状态良好,无肿瘤局部复发和转移征象。结论经典肾母细胞瘤成分和肿瘤细胞表达WT1阳性对诊断和鉴别诊断至关重要。该瘤通常不具有侵袭力,手术完整切除后预后较好。

Objective To study the histopathologic features,differential diagnosis and prognosis of teratoid Wilms＇ tumor.Methods A case of teratoid Wilms＇ tumor were retrieved from the records of Department of Pathology,Children＇s Hospital of Jiaotong University.The clinicopathologic features,immunohistochemistry,ultrastructural findings and follow-up data were studied and literature reviewed.Results Histologically,the tumor consisted predominantly of heterologous tissues（lake of kerator,adipose,muscle,cartilage,or lace-like osteoid） and islands of classic Wilms＇ tumor＇s three components（blastemal,primive tubuli and stromal）.Immunohistochemical study showed that tumor cells were positive for WT1.During follow-up,patients remained well and disease-free for 26 months after operation.Conclusions Triphasic pattern of classic Wilms＇ tumor and positive WT1 found in tumor cells is very important for diagnosis and differential diagnosis.This kind of tumor usually is not aggressive,and the outcome is good.Surgical treatment produces excellent prognosis.