Hase Takanobu, Ohta Shigeru, Tani Tohru, Mizukuro Tomoyuki, Mekata Eizi, Naitoh Hiroyuki, Shimadera Shin'ichi, Fujino Syouzou, Taga Takashi
Department of Emergency and Critical Medicine/Medical Coordination Center, Shiga University of Medical Science, Tsukinowa-cho, Otsu, Shiga, 520-2192 Japan.
Pediatr Surg Int. 2002 Sep;18(5-6):289-94. doi: 10.1007/s003830100701. Epub 2002 Aug 2.
To investigate retrospectively the clinical and biological features that influence the outcome of infants with neuroblastoma (NB) detected by mass screening (NBMS), and to construct surgical strategies to deal with NBMS, 20 infants diagnosed as having either NB or ganglioneuroblastoma (GNB) between 1986 and 1998 were enrolled in a study. They comprised 15 boys and 5 girls ranging in age from 7 to 14 months. The following factors were analyzed by multivariate analysis: age, stage according to the Japanese staging system at the time of diagnosis, site of the primary tumor, histologic findings, preoperative urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels, VMA/HVA ratio, lactate dehydrogenase, neuron-specific enolase, Shimada's histologic classification, amplification of the N- myc oncogene by Southern blot analysis, nuclear content, and chromosomal abnormality. The 2-year survival was 95.0% (19/20). The site of the primary tumor was the adrenal gland in 12 cases, retroperitoneum in 6, and retrothoracic space in 2. Four infants had stage I, 6 stage II, 3 stage III, 3 stage IVB, and 4 stage IV disease. Complete resection was achieved in all cases except for 1 retroperitoneal GNB. Histologic examination showed that 8 patients had NB. Of the 12 GNBs, 8 were poorly-differentiated and 1 was well-differentiated. Only 1 of the 14 examined tumors showed amplification of N- myc (20 copies). The infant with N- myc-positive NB (stage II) died 23 months after surgery in spite of aggressive postoperative chemotherapy. Multivariate analysis revealed the plasma NSE level to be a significant predictor of survival (p < 0.0143). This suggests that N- myc amplification and plasma NSE level could be closely related to the survival of infants with NBMS. The N- myc-positive NB case implies that even in locoregional NB detected by NBMS, surgical excision should play a central role in the diagnosis of its oncogenic characteristics and indicate any subsequent therapy.
为回顾性研究影响通过大规模筛查（NBMS）检测出的神经母细胞瘤（NB）患儿预后的临床和生物学特征，并构建应对NBMS的手术策略，1986年至1998年间诊断为NB或神经节神经母细胞瘤（GNB）的20例婴儿被纳入一项研究。他们包括15名男孩和5名女孩，年龄在7至14个月之间。通过多变量分析对以下因素进行分析：年龄、诊断时根据日本分期系统的分期、原发肿瘤部位、组织学发现、术前尿香草扁桃酸（VMA）和高香草酸（HVA）水平、VMA/HVA比值、乳酸脱氢酶、神经元特异性烯醇化酶、岛田组织学分类、通过Southern印迹分析检测的N-myc癌基因扩增、核含量和染色体异常。2年生存率为95.0%（19/20）。原发肿瘤部位：肾上腺12例，腹膜后6例，后纵隔2例。4例婴儿为Ⅰ期，6例为Ⅱ期，3例为Ⅲ期，3例为ⅣB期，4例为Ⅳ期。除1例腹膜后GNB外，所有病例均实现了完整切除。组织学检查显示8例患者为NB。在12例GNB中，8例为低分化，1例为高分化。在14例检测的肿瘤中，仅1例显示N-myc扩增（20拷贝）。尽管术后进行了积极化疗，N-myc阳性NB（Ⅱ期）婴儿术后23个月死亡。多变量分析显示血浆NSE水平是生存的显著预测指标（p<0.0143）。这表明N-myc扩增和血浆NSE水平可能与NBMS婴儿的生存密切相关。N-myc阳性NB病例表明，即使在通过NBMS检测出的局限性NB中，手术切除在其致癌特征的诊断中也应发挥核心作用，并为后续治疗提供指导。
