The nephroblastomatosis complex and its relationship to Wilms' tumor: a clinicopathologic treatise.
The nephroblastomatosis complex and its relationship to Wilms' tumor: a clinicopathologic treatise.
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肾母细胞瘤病综合征及其与肾母细胞瘤的关系：临床病理学论文。
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发表时间：
1976
期刊：
影响因子：
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通讯作者：
A. J. Mcadams
中科院分区：
文献类型：
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作者：
K. Bove;A. J. Mcadams
1.至少有三分之一的肾母细胞瘤患儿有后肾分化异常、肾包膜下错构瘤、腺瘤和芽基结节或肾母细胞瘤。只有结节性肾芽基的存在被发现与年轻年龄相关，这表明其他肿瘤样病变可能是衍生物。2.在我们的经验中，所有双侧和连续双侧肾母细胞瘤患者在肾切除术中切除的“未受累”肾组织都表现出这些特征。3.肾母细胞瘤病的全皮质（婴儿），弥漫性浅表（晚期婴儿）和多灶性（青少年）变种的定义。在后两种形式的肾母细胞瘤病的现代治疗后的预后至少是一样好，因为它是在一个组织学正常的肾脏发生单侧，单中心的肾母细胞瘤的患者。4.形态学证据有力地表明肾母细胞瘤病患者的肾母细胞瘤是由结节性芽基或后肾错构瘤发展而来。假设所有的肾母细胞瘤都是从这些前体细胞发展而来的。肾发生停止和肾母细胞瘤临床表现之间的潜伏期被解释为支持这样的假设，即癌发生本身是出生后事件的结果，但肿瘤发展的底物，异常后肾增殖，在妊娠期间开始。
1. At least one third of all children with Wilms' tumors have evidence of aberrant metanephric differentiation, hamartomas, adenomas and nodules of blastema or Wilms' tumorlets in the subcapsular renal cortex. Only the presence of nodular renal blastema was found to correlate with younger age, suggesting that the other tumorlike lesions may be derivatives. 2. In our experience, all patients with bilateral and sequential bilateral Wilms' tumors have exhibited these characteristics in the "uninvolved" renal tissue removed at nephrectomy. 3. Pancortical (infantile), diffuse superficial (late infantile) and multifocal (juvenile) variants of nephroblastomatosis are defined. In the latter 2 forms of nephroblastomatosis the prognosis after modern therapy is at least as good as it is in patients with unilateral, unicentric Wilms' tumor arising in a histologically normal kidney. 4. The morphologic evidence presented strongly indicates that Wilms' tumor in patients with nephroblastomatosis develops either from nodular blastema or metanephric hamartomas. It is hypothetically possible that all Wilms' tumors develop from these precursors. The latent period between cessation of nephrogenesis and clinical presentation of Wilms' tumor is interpreted as favoring the hypothesis that carcinogenesis per se is the result of a postnatal event but that the substrate for tumor development, aberrant metanephric proliferation, has its inception during gestation.