Matthay K K, Sather H N, Seeger R C, Haase G M, Hammond G D
University of California School of Medicine, San Francisco.
J Clin Oncol. 1989 Feb;7(2):236-44. doi: 10.1200/JCO.1989.7.2.236.
The optimal management for patients with stage II neuroblastoma has not yet been established. In order to determine the impact of adding chemotherapy and/or radiation therapy to surgery, we reviewed by questionnaire 156 patients with stage II neuroblastoma treated by 28 Childrens Cancer Study Group (CCSG) institutions from 1978 to 1985. Survival and progression-free survival (PFS) were analyzed by life-table methods with respect to age at diagnosis, site and size of primary tumor, spinal cord involvement, extent of initial resection, and treatment in addition to surgery. The overall 5-year survival was 96%; the PFS was 90%, similar to previous CCSG studies. Age at diagnosis had a small impact on PFS, with 92% PFS for patients less than 2 years of age at diagnosis, and 84% for those greater than 2 (P = .10). The only site with an adverse outcome was the head and neck (n = 11), with a PFS of 68% compared with 93% for the remaining sites (P = .02). Size of primary and intraspinal extension of primary did not affect PFS. The extent of resection and subsequent treatment with radiation therapy and/or chemotherapy did not affect the PFS. The outcome for 75 patients treated with surgery alone (6-year PFS, 89%) was not significantly different from that of 66 patients receiving radiation therapy (6-year PFS, 94%). There was no significant difference between 40 patients with gross or microscopic residual disease treated with surgery alone (PFS, 92%) and 59 patients with residual disease who also received radiation (PFS, 90%). Five of seven patients who progressed after surgery alone have been salvaged with further therapy and are now free of disease. One survives with disease, so that the 6-year survival is 98% for those treated initially with surgery alone, compared with 95% for those receiving radiation therapy and/or chemotherapy. These data suggest that surgery alone, even if complete resection is not achieved, is sufficient initial therapy for stage II neuroblastoma. The data also identify another stage of neuroblastoma, in addition to stage IV-S, for which almost all patients have a favorable prognosis because their tumor may be biologically limited in growth.
II期神经母细胞瘤患者的最佳治疗方案尚未确定。为了确定在手术基础上增加化疗和/或放疗的影响，我们通过问卷调查回顾了1978年至1985年期间由28个儿童癌症研究组（CCSG）机构治疗的156例II期神经母细胞瘤患者。采用寿命表法分析了诊断时的年龄、原发肿瘤的部位和大小、脊髓受累情况、初始切除范围以及除手术外的治疗对生存率和无进展生存期（PFS）的影响。总体5年生存率为96%；无进展生存期为90%，与CCSG之前的研究相似。诊断时的年龄对无进展生存期有较小影响，诊断时年龄小于2岁的患者无进展生存期为92%，大于2岁的患者为84%（P = 0.10）。唯一预后不良的部位是头颈部（n = 11），无进展生存期为68%，其余部位为93%（P = 0.02）。原发肿瘤大小和原发肿瘤的脊髓内扩展不影响无进展生存期。切除范围以及随后的放疗和/或化疗治疗均不影响无进展生存期。75例仅接受手术治疗的患者（6年无进展生存期，89%）与66例接受放疗的患者（6年无进展生存期，94%）的预后无显著差异。40例仅接受手术治疗的有大体或镜下残留病灶的患者（无进展生存期，92%）与59例有残留病灶且也接受放疗的患者（无进展生存期，90%）之间无显著差异。7例仅接受手术后病情进展的患者中有5例通过进一步治疗得到挽救，目前无病生存。1例带瘤生存，因此，最初仅接受手术治疗的患者6年生存率为98%，接受放疗和/或化疗的患者为95%。这些数据表明，即使未实现完全切除，单纯手术也是II期神经母细胞瘤足够的初始治疗。这些数据还确定了神经母细胞瘤的另一个阶段，除了IV-S期，几乎所有患者的预后都良好，因为他们的肿瘤在生物学上生长可能受限。
