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肾母细胞瘤的临床病理特征

Clinicopathological profile of Wilms' tumor.

作者信息

Paul L, Thaver D, Muzaffar S, Soomro I N, Nazir Z, Hasan S H

机构信息

Department of Pathology and Surgery, Aga Khan University Hospital, Karachi, Pakistan.

出版信息

Indian J Pediatr. 2000 Oct;67(10):765-7. doi: 10.1007/BF02723937.

Abstract

The profile of renal tumors in children less than 15 years of age during the period 1991-1997 is presented. Among the 37 children with kidney tumors, 29 (78.4%) had Wilms' tumor. There was also a 20-year-old female with Wilms' tumor. The median age at presentation was 2.6 years (range 2.5 months to 20 years). 66.7% of the cases diagnosed were < or = 3 years and 90% were < or = 6 years. Five cases were under one year of age. The male to female ratio was 2:1. Twenty-two cases (73.3%) were triphasic and 7 (23.3%) were biphasic. Only one case was monophasic with blastemal component. Five cases (16.7%) showed nephrogenic rests in the uninvolved renal parenchyma and one case had nephroblastomatosis. The tumor was favorable in 26 cases (86.7%) and unfavorable in 4. Fourteen cases were in-patients while 16 were outside referrals. The pathological (10 cases whose specimens were sent from other centers) and clinicopathological (13 hospitalized patients) staging showed 10 cases (43.5%) with stage 1, 4 cases (17.4%) with stage 2, and 7 cases (30.4%) with stage 3. In two cases (8.7%), there was stage 4 disease. The length of the follow-up period in the 13 hospitalized patients ranged from 7 days to 5 years 5 months (median 14 months). There was one recurrence and one death after 2 years of diagnosis.

摘要

本文呈现了1991年至1997年期间15岁以下儿童肾肿瘤的概况。在37例肾肿瘤患儿中,29例(78.4%)患有肾母细胞瘤。还有一名20岁的女性肾母细胞瘤患者。发病时的中位年龄为2.6岁(范围为2.5个月至20岁)。66.7%的确诊病例年龄≤3岁,90%的病例年龄≤6岁。5例患儿年龄在1岁以下。男女比例为2:1。22例(73.3%)为三相型,7例(23.3%)为双相型。仅1例为含胚基成分的单相型。5例(16.7%)在未受累肾实质中显示肾源性残留,1例患有肾母细胞瘤病。26例(86.7%)肿瘤预后良好,4例预后不良。14例为住院患者,16例为外院转诊患者。病理分期(10例标本来自其他中心)和临床病理分期(13例住院患者)显示,10例(43.5%)为1期,4例(17.4%)为2期,7例(30.4%)为3期。2例(8.7%)为4期疾病。13例住院患者的随访期为7天至5年5个月(中位值为14个月)。确诊2年后有1例复发和1例死亡。

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