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神经母细胞瘤:原发性肿瘤不完全切除后的治疗结果

Neuroblastoma: treatment outcome after incomplete resection of primary tumors.

作者信息

Moon Suk-Bae, Park Kwi-Won, Jung Sung-Eun, Youn Woong-Jae

机构信息

Department of Pediatric Surgery, Seoul National University Children's Hospital, Seoul, Korea.

出版信息

Pediatr Surg Int. 2009 Sep;25(9):789-93. doi: 10.1007/s00383-009-2417-8. Epub 2009 Jul 21.

Abstract

PURPOSE

For International Neuroblastoma Staging System (INSS) stages III or IV neuroblastoma (intermediate or high risk), complete excision of the primary tumor is not always feasible. Most current studies on the treatment outcome of these patients have reported on the complete excision status. The aim of this study is to review the treatment outcome after the incomplete resection.

METHODS

The medical records of 37 patients that underwent incomplete resection between January 1986 and December 2005 were reviewed retrospectively. Incomplete resection was assessed by review of the operative notes and postoperative computerized tomography. Age, gender, tumor location, INSS stage, N-myc gene copy number, pre- and postoperative therapy, and treatment outcome were reviewed. The treatment outcome was evaluated according to the postoperative treatment protocol in the high-risk group.

RESULTS

Intermediate-risk patients were treated with conventional chemotherapy, isotretinoin (ITT) and interleukin-2 (IL-2). High-risk patients were treated with peripheral blood stem cell transplantation (PBSCT), ITT, and IL-2 (N = 11). Before the introduction of PBSCT, the high-risk patients were also treated with the conventional chemotherapy (N = 19). Intermediate-risk patients (N = 5) currently have no evidence of disease (NED). For the high-risk patients (N = 32), 19 patients were treated with chemotherapy alone; 15 patients died of their disease while four patients currently have an NED status. Eight of 11 patients that underwent PBSCT are currently alive.

CONCLUSIONS

For intermediate risk, conventional chemotherapy appears to be acceptable treatment. However, for high-risk patients, every effort should be made to control residual disease including the use of myeloablative chemotherapy, differentiating agents and immune-modulating agents.

摘要

目的

对于国际神经母细胞瘤分期系统(INSS)Ⅲ期或Ⅳ期神经母细胞瘤(中危或高危),完整切除原发肿瘤并非总是可行的。目前大多数关于这些患者治疗结果的研究报告的是完整切除情况。本研究的目的是回顾不完全切除后的治疗结果。

方法

回顾性分析1986年1月至2005年12月期间接受不完全切除的37例患者的病历。通过查阅手术记录和术后计算机断层扫描评估不完全切除情况。回顾了年龄、性别、肿瘤位置、INSS分期、N - myc基因拷贝数、术前和术后治疗以及治疗结果。根据高危组术后治疗方案评估治疗结果。

结果

中危患者接受传统化疗、异维A酸(ITT)和白细胞介素-2(IL - 2)治疗。高危患者接受外周血干细胞移植(PBSCT)、ITT和IL - 2治疗(n = 11)。在引入PBSCT之前,高危患者也接受传统化疗(n = 19)。中危患者(n = 5)目前无疾病证据(NED)。对于高危患者(n = 32),19例患者仅接受化疗;15例患者死于疾病,4例患者目前处于NED状态。11例接受PBSCT的患者中有8例目前存活。

结论

对于中危患者,传统化疗似乎是可接受的治疗方法。然而,对于高危患者,应尽一切努力控制残留疾病,包括使用清髓性化疗、分化剂和免疫调节剂。

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