Rhabdomyogenesis in renal neoplasia of childhood
Rhabdomyogenesis in renal neoplasia of childhood
复制标题
儿童肾肿瘤中的横纹肌发生
DOI：
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发表时间：
1981
影响因子：
5.6
通讯作者：
N. Ugarte
中科院分区：
文献类型：
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作者：
F. Gonzalez‐Crussi;W. Hsueh;N. Ugarte
在 220 个连续的儿童原发性肾肿瘤中，17 个含有大量组织学上可识别的横纹肌细胞（超过采样肿瘤实质的 10%）。这些肿瘤可以进一步分为两组：具有“大量”横纹肌生成的维尔姆斯氏肿瘤（肿瘤实质的三分之一或更多由肌肉组成）和具有“中度”横纹肌生成的维尔姆斯氏肿瘤（10-30％的肌肉成分）。前一种肿瘤总是见于4岁以下的幼儿；通常患者是1岁或更小的婴儿，并且该组中超过一半的患者患有双侧横纹肌增生。患有“中度”横纹肌生成肿瘤的患者均未发现双侧性肿瘤，此外，两组患者均存在盆腔内息肉样生长的倾向，除其中一个肿瘤外，所有肿瘤均被归类为肾母细胞瘤；唯一的例外是患有与肾母细胞瘤相关的先天性畸形的患者。然而，两组中都存在与肿瘤性多灶性起源一致的解剖学病变，因此，我们的研究结果表明，广泛的横纹肌发生与临床行为之间存在明确的相关性，这与将这种组织学变异视为肾母细胞瘤的细胞分化形式充分一致。
Of 220 consecutive primary renal tumors of childhood, 17 contained substantial amounts of histologically identifiable striated muscle cells (over 10% of sampled tumor parenchyma). These tumors could be further subclassified into two groups: Wilms' tumors with “massive” rhabdomyogenesis (one-third or more of the tumor parenchyma composed of muscle), and Wilms' tumors with “moderate” rhabdomyogenesis (10–30% muscle composition. The former tumors were invariably seen in young children, under 4 years of age; often the patients were infants 1 year of age, or younger, and more than half of the patients in this group had bilateral tumors. Bilaterality was not seen in patients harboring tumors with “moderate” rhabdomyogenesis, who, in addition, were older children. In both groups, there was a tendency for polypoid intrapelvic growth. All but one of the tumors described in this report were classified as Wilms' tumor; the single exception was considered to be a primary rhabdomyosarcoma of the kidney. Patients with congenital malformations related to Wilms' tumor (one aniridia, one hemihypertrophy) were seen only in the group with “massive” rhabdomyogenesis. However, anatomical lesions consistent with neoplastic multifocal orgin were present in both groups. Thus, our findings indicate a definite correlation between extensive rhabdomyogenensis and clinical behavior. This relation is expressed in patterns of age distribution, bilaterality and manner of growth, which are sufficiently consistent to individualize this histologic variant as a cytodifferentiated form of nephroblastoma.