Okazaki Tadaharu, Kohno Sumio, Mimaya Jun-ichi, Hasegawa Shiro, Urushihara Naoto, Yoshida Atsushi, Kawano Shinya, Kusafuka Junichi, Horikoshi Yasuo, Takashima Yoshifumi, Aoki Katsuhiko, Hamazaki Minoru
Department of Pediatric Surgery, Shizuoka Children's Hospital, 860 Urushiyama, 420-8660, Shizuoka, Japan.
Pediatr Surg Int. 2004 Jan;20(1):27-32. doi: 10.1007/s00383-003-1070-x. Epub 2003 Dec 19.
Japan has a nationwide mass-screening program for neuroblastoma in 6-month-old infants. Neuroblastoma can regress spontaneously, and some institutions observe selected cases. We evaluated the management of screened neuroblastoma at our hospital since 1997 when an observation program was introduced. Criteria for the observation program were stage-I, stage-II, or stage-IVs tumors, urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels <40 microg/mg creatinine, tumor <5 cm in diameter, no invasion to the intraspinal canal or great vessels, and parental consent to participate. Patients who did not meet observation criteria underwent surgery or mild chemotherapy according to the location of the tumor. If patients met observation criteria after chemotherapy, surgical intervention was no longer performed. Thirty-six patients attended our hospital for screened neuroblastoma from 1997 to 2002. Thirty-three patients who were managed at our hospital participated in this study. Ten subjects met observation criteria. Tumors regressed in 7 patients (mean follow-up period 36.3 months) with corresponding decreases in VMA and HVA levels (group A). Three underwent surgery (group B) because of increasing VMA and HVA levels, increase in tumor size, or guardian's request. Twenty-three subjects did not meet observation criteria. Four patients underwent primary surgery (group C), and 19 patients had chemotherapy initially. Fourteen patients met observation criteria after chemotherapy and two are still having chemotherapy (group D). Three patients required surgery due to insufficient regression of their tumors (group E). Fourteen subjects in group D had marked decreases in VMA and HVA levels and tumor size (mean follow-up period 29.1 months), and tumors were not detected using imaging techniques in 8 patients. Histological examination of all resected specimens during the study period showed favorable histology and no N-myc amplification. There was no evidence of unfavorable prognosis in any of the 33 subjects, although 1 patient who underwent primary surgery had a vanishing kidney 1 year later and 1 patient had multiple bony metastases after complete resection of tumor, which was treated by chemotherapy. Until the real significance of mass screening for neuroblastoma as a public health measure is confirmed, observation with careful follow-up should be adopted more extensively because it has a favorable outcome in many cases, and is associated with minimal therapeutic complications.
日本针对6个月大的婴儿开展了全国性的神经母细胞瘤大规模筛查项目。神经母细胞瘤可自发消退,一些机构对部分病例进行观察。自1997年引入观察项目以来,我们评估了本院对筛查出的神经母细胞瘤的管理情况。观察项目的标准为I期、II期或IVs期肿瘤,尿香草扁桃酸(VMA)和高香草酸(HVA)水平<40μg/mg肌酐,肿瘤直径<5cm,无椎管或大血管侵犯,且父母同意参与。不符合观察标准的患者根据肿瘤位置接受手术或轻度化疗。若患者化疗后符合观察标准,则不再进行手术干预。1997年至2002年期间,有36例因筛查出神经母细胞瘤来我院就诊。在我院接受管理的33例患者参与了本研究。10例符合观察标准。7例患者的肿瘤消退(平均随访期36.3个月),VMA和HVA水平相应下降(A组)。3例因VMA和HVA水平升高、肿瘤增大或监护人要求而接受手术(B组)。23例不符合观察标准。4例患者接受了初次手术(C组),19例患者最初接受化疗。14例患者化疗后符合观察标准,2例仍在接受化疗(D组)。3例患者因肿瘤消退不充分而需要手术(E组)。D组的14例患者VMA和HVA水平及肿瘤大小显著下降(平均随访期29.1个月),8例患者经影像学检查未发现肿瘤。研究期间所有切除标本的组织学检查均显示组织学良好且无N - myc扩增。33例患者中均无不良预后的证据,尽管1例接受初次手术的患者1年后出现患肾消失,1例患者在肿瘤完全切除后出现多处骨转移,经化疗治疗。在神经母细胞瘤大规模筛查作为一项公共卫生措施的真正意义得到证实之前,应更广泛地采用密切随访观察,因为在许多情况下其结果良好,且治疗并发症极少。