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肾母细胞瘤病和多发性双侧肾母细胞瘤。组织学、治疗及理论方面

Nephroblastomatosis and multiple bilateral nephroblastomata. Histologic, therapeutic, and theoretical aspects.

作者信息

Machin G A

出版信息

Arch Pathol Lab Med. 1978 Dec;102(12):639-42.

PMID:214054
Abstract

A hemihypertrophic girl had multiple sequential bilateral Wilms' tumors over a period of five years. Diagnostic biopsy and resected specimens showed also the presence of nephroblastomatosis, presumably the substrate from which these multiple tumors arose. The patient died of complications of chemotherapy and dialysis after bilateral nephrectomy, but also had residual tumor. The relationships between nephroblastomatosis, Wilms' tumor, bilateral Wilms' tumor, and teratogenic disorders are discussed, and the therapeutic implications of the nephroblastomatosis/nephroblastoma complex are considered.

摘要

一名半身肥大的女孩在五年间先后出现双侧肾母细胞瘤。诊断性活检及切除标本显示也存在肾母细胞瘤病,推测其为这些多发肿瘤的起源基质。患者在双侧肾切除术后死于化疗及透析并发症,但仍有残留肿瘤。文中讨论了肾母细胞瘤病、肾母细胞瘤、双侧肾母细胞瘤及致畸性疾病之间的关系,并考虑了肾母细胞瘤病/肾母细胞瘤复合体的治疗意义。

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