Stay E J, Vawter G
Cancer. 1977 Jun;39(6):2550-5. doi: 10.1002/1097-0142(197706)39:6<2550::aid-cncr2820390636>3.0.co;2-y.
Three cases with co-existent Wilm's tumor and Von Recklinghausen's Disease from a series of 342 nephroblastomas are discussed as to the significance of this association. Biological arguments favor a positive link because of the multiplicity of associative lesions which parallel each other, the co-existence of which suggests a common genetic bond. An embryologic association is described whereby early mutational events, i.e. neural induction of nephrogenesis, may link these two entities in utero. Further interconnection is stated statistically, in that nephroblastoma patients in this series had a 29 fold higher incidence of multiple neurofibromatosis than predicted for in the general population. The co-existent diseases in the three cases observed are approximately 33-50% more than can be accounted for by chance in the United States population. Malignant transformation in neurofibromatosis is discussed because of the therapeutic implications presented by two of the three Wilms' tumor patients studied.
在342例肾母细胞瘤病例中，有3例同时存在肾母细胞瘤和冯雷克林霍增氏病，本文讨论了这种关联的意义。基于生物学的论据支持两者存在正向联系，因为相关病变具有多样性且相互平行，它们的共存表明存在共同的基因纽带。本文描述了一种胚胎学上的关联，即早期的突变事件，也就是肾发生的神经诱导，可能在子宫内将这两种疾病联系起来。从统计学角度进一步阐述了两者的关联性，该系列研究中的肾母细胞瘤患者患多发性神经纤维瘤病的几率比普通人群预测的高出29倍。在美国人群中，观察到的这3例病例中同时存在这两种疾病的情况比偶然情况多出约33% - 50%。鉴于所研究的3例肾母细胞瘤患者中有2例出现了具有治疗意义的情况，因此对神经纤维瘤病的恶性转化进行了讨论。
