Halperin E C
Duke Comprehensive Cancer Center, Duke University Medical Center, Durham, North Carolina, USA.
J Surg Oncol. 1996 Nov;63(3):172-8. doi: 10.1002/(SICI)1096-9098(199611)63:3<172::AID-JSO7>3.0.CO;2-A.
The appropriate therapy for Stage C neuroblastoma (NB) is uncertain. Because of the need for information applicable to the development of new randomized trials, we deemed it appropriate to investigate the patient characteristics, survival, patterns of failure, and complications of therapy in these children.
Search of the medical records of Duke University Medical Center from 1/1/60 to 3/1/95 disclosed 146 patients with NB, which included 13 Stage C patients.
Mean age at diagnosis was 3.6 years. Twelve patients had primary abdominal tumors (92%) and one had a thoracic primary (8%). Twelve (92%) of the patients received chemotherapy including cyclophosphamide. 11 (85%). Adriamycin, 6 (46%), cisplatinum, 4 (30%), and VP 16, 4 (30%). All patients received radiotherapy (RT, mean dose administered 22.6 +/- 8 Gy). With a mean follow-up of 8 years, the 10-year overall survival was 54% and the relapse-free survival was 46%. Four patients relapsed in the primary operative tumor bed and primary RT field, two relapsed in mediastinal or left supraclavicular lymph nodes as well as distantly following treatment of upper abdominal primaries, and in one the site of relapse is unknown. Long-term complications of therapy included two children who developed secondary malignancies associated with RT, two girls who developed primary ovarian failure, five children with clinically significant kyphosis and scoliosis, and one who suffered postoperative wound dehiscence following RT.
Although this study did not include modern techniques of staging with n-myc amplification and DNA index, the occurrence of next echelon nodal failures gives credence to the continuation of the dialogue concerning the appropriate role of "prophylactic" irradiation to mediastinal and left supraclavicular nodes in locally advanced upper abdominal NB. Documentation of significant long-term ill effects reinforces the need to critically evaluate the indications for RT.
C期神经母细胞瘤（NB）的合适治疗方法尚不确定。由于需要适用于新随机试验开展的信息，我们认为对这些儿童的患者特征、生存率、失败模式及治疗并发症进行研究是恰当的。
检索杜克大学医学中心1960年1月1日至1995年3月1日的病历，发现146例NB患者，其中包括13例C期患者。
诊断时的平均年龄为3.6岁。12例患者有原发性腹部肿瘤（92%），1例有原发性胸部肿瘤（8%）。12例（92%）患者接受了化疗，包括环磷酰胺。11例（85%）接受阿霉素，6例（46%）接受顺铂，4例（30%）接受VP 16，4例（30%）接受VP 16。所有患者均接受了放疗（RT，平均给予剂量22.6±8 Gy）。平均随访8年，10年总生存率为54%，无复发生存率为46%。4例患者在原发手术肿瘤床和原发放疗区域复发，2例在纵隔或左锁骨上淋巴结复发，以及在上腹部原发肿瘤治疗后远处复发，1例复发部位不明。治疗的长期并发症包括2例发生与放疗相关的继发性恶性肿瘤的儿童，2例发生原发性卵巢功能衰竭的女孩，5例有临床显著驼背和脊柱侧弯的儿童，以及1例放疗后术后伤口裂开的患者。
尽管本研究未包括采用n - myc扩增和DNA指数进行分期的现代技术，但下一级淋巴结失败的发生为继续讨论“预防性”照射纵隔和左锁骨上淋巴结在局部晚期上腹部NB中的适当作用提供了依据。显著长期不良影响的记录强化了严格评估放疗适应证的必要性。
