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[胎儿横纹肌瘤样肾母细胞瘤:2例病例研究及文献复习]

[Fetal rhabdomyomatous nephroblastoma: study of 2 cases and review of the literature].

作者信息

Vicandi B, Picazo M L, González M C, Contreras F

出版信息

An Esp Pediatr. 1985 Sep;23(3):205-10.

PMID:3000239
Abstract

Report of two cases of Fetal Rhabdomyomatous Nephroblastoma, a cytodifferentiated variant of Wilms' tumor. The incidence of this rare variant is of 2.10% in our material (88 nephroblastomas in a sixteen years period). The patients were ten months and two years old. Bilateral tumors were discovered in both patients. Preoperative radiotherapy was given but no reduction of the tumors size was obtained. Microscopically, benign looking striated muscle is the predominant component of the tumors, with scare foci of epithelial elements. The low age of presentation, high incidence of bilaterality and the peculiar intrapelvic renal growth are three relevant characteristics in the 35 cases previously reported as well as in these two new cases, that distinguish Fetal Rhabdomyomatous Nephroblastoma from conventional Wilms' tumor.

摘要

胎儿横纹肌瘤样肾母细胞瘤(一种肾母细胞瘤的细胞分化变异型)两例报告。在我们的资料中(16年间88例肾母细胞瘤),这种罕见变异型的发生率为2.10%。患者分别为10个月和2岁。两名患者均发现双侧肿瘤。术前进行了放疗,但肿瘤大小未缩小。显微镜下,外观良性的横纹肌是肿瘤的主要成分,上皮成分灶性少见。在先前报道的35例以及这两例新病例中,发病年龄低、双侧发生率高以及肾盂内肾脏生长特殊是三个相关特征,这些特征将胎儿横纹肌瘤样肾母细胞瘤与传统肾母细胞瘤区分开来。

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