神経芽腫 IV-S 症例の遺伝学的背景 : Knudson 理論の検証

概要

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Genetic background of neuroblastoma IV-S was studied, based upon the data on 380 cases of neuroblastomata, collected from 11 main facilities in Japan. Among 380 cases collected, 16 belonged to the group neuroblastoma IV-S. All the IV-S cases had the onset before 12 months. This is remarkably earlier than any other stages of this disease. There was no case with multiple origin of the tumor in this collected series of IV-S. But one case of IV-S with bilateral adrenal origin was experienced in the Pediatric Surgical Department of Tsukuba University, and moreover there were some reports on multiple origin in the literature. The familial occurrence rate of neuroblastoma IV-S was 1/16 (6.3%), while that of other stages were 1/364 (0.3%). The young onset and multiplicity, which were demonstrated as two important characteristics of familial tumor by Knudson, were well proven in the neuroblastoma IV-S in this study.
日本小児外科学会の論文