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成髓细胞瘤成人和儿童的治疗结果:美国国立癌症研究所、开罗大学的经验

Treatment results of adults and children with medulloblastoma NCI, Cairo University experience.

作者信息

Khalil Ehab M

机构信息

The Department of Radiotherapy and Nuclear Medicine, National Cancer Institute, Cairo University.

出版信息

J Egypt Natl Canc Inst. 2008 Jun;20(2):175-86.

PMID:20029474
Abstract

PURPOSE

To evaluate treatment outcome and prognostic factors of adults and pediatric medulloblastoma patients treated by adjuvant postoperative craniospinal irradiation (CSI) and chemotherapy.

PATIENTS AND METHODS

Between 1997 and 2004, 67 patients were treated in the National cancer Institute- Cairo University; 51 pediatric patients with a median age of 7 years and 16 adult patients with a median age of 25 years. According to the Chang staging system; 50%-35% , 37.5%-47% and 12.5%-18% had T2, T3 and T4 tumors of adults and pediatric patient's population respectively. M1, M2 and M4 were reported in 16% , 3% and in 1.5% respectively. All patients underwent primary surgical resection; near total resection in 25% , Subtotal resection in 61% ; with tumor residual < 1.5cm(2) in 49% compared to 51% with > 1.5cm(2) residual tumor and 14% , had biopsy only. All patients were treated by craniospinal radiotherapy (RT); with a median dose of 34Gy to the whole brain, 54Gy to the posterior fossa and 32Gy to the spinal axis. The median interval between surgery and RT was 45 days and 38 days for the pediatric and adult groups respectively. The median duration of RT was 54 days and 52 days for pediatric and adult patients respectively. Thirty four pediatric patients (67% ) received concomitant chemotherapy, while 61% received adjuvant (postoperative) chemotherapy and 57% received sequential chemotherapy. Only 33% of patients did not receive chemotherapy. The median follow-up was 49 months for the whole group of patients (range 11-121).

RESULTS

For the pediatric and adult patients, the 5- and 7-year overall and disease-free survival rates were 89% & 78% Vs. 84% & 56% and 80% & 68% Vs. 79% & 52% respectively. Fourteen patients (21% ) relapsed (10 pediatric and 4 adults) at a median time of 11 months vs. 23 months and a median follow-up period of 8 and 12 months respectively; Neuro-axis was the most common site of relapse (11 patients). Ninety percent (9/10) of the pediatric relapses were of the high risk group (8 received no chemotherapy) and took place within 2 years; similarly all adult relapses were of the high risk group; three relapses took place after 2 years. In univariate analysis, T stages, M stages, extent of surgery, CSF seedling and risk category were significant prognostic factors for disease free survival for the pediatric age group. At 5 years, 87% , 87% , 78% , 85% and 85% of T2, M0, near total resection, negative CSF seedling and low risk patients were estimated to be disease-free respectively, compared to none of the T4, M +, biopsy only, positive CSF seedling patients and only 41% of the high risk patients achieved 5 years without recurrence (p=0.001, <0.001, <0.001, <0.001, <0.001). Patients who did not receive chemotherapy had a 69% 5-year DFS Vs. 76% (p=0.286). For adult patients; only the risk category was a significant prognostic factor with 5-year disease-free survival rate of 100% Vs. 40% for low and high risk respectively (p=0.03). On multivaiate analysis only the risk category and the T-stage were significant prognostic factors for disease free survival for the pediatric age group (p=0.042 and 0.031).

CONCLUSION

Survival rates of medulloblastoma pediatric patients were better than the adult ones. Neuro-axis relapse was the most common site of relapse for pediatric patients. Late relapses, lateral tumor location and shorter median follow up were noted in adult patients. Advanced tumor stage, metastases at presentation, limited tumor resection were powerful prognostic factors among the pediatric patients. In addition, high risk category was shown to be a prognostic factor for both pediatric and adult patients.

摘要

目的

评估接受辅助性术后颅脊髓放疗(CSI)和化疗的成髓细胞瘤成年和儿童患者的治疗效果及预后因素。

患者与方法

1997年至2004年间,开罗大学国家癌症研究所治疗了67例患者;51例儿童患者,中位年龄7岁,16例成年患者,中位年龄25岁。根据Chang分期系统;成年和儿童患者群体中分别有50%-35%、37.5%-47%和12.5%-18%患有T2、T3和T4期肿瘤。分别有16%、3%和1.5%的患者报告为M1、M2和M4期。所有患者均接受了初次手术切除;25%为近全切除,61%为次全切除;49%的患者肿瘤残留<1.5cm²,51%的患者肿瘤残留>1.5cm²,14%的患者仅接受了活检。所有患者均接受了颅脊髓放疗(RT);全脑中位剂量为34Gy,后颅窝为54Gy,脊髓轴为32Gy。儿童和成年组手术与放疗的中位间隔分别为45天和38天。儿童和成年患者放疗的中位持续时间分别为54天和52天。34例儿童患者(67%)接受了同步化疗,61%接受了辅助(术后)化疗,57%接受了序贯化疗。仅33%的患者未接受化疗。全组患者的中位随访时间为49个月(范围11-121个月)。

结果

对于儿童和成年患者,5年和7年的总生存率及无病生存率分别为89%和78%对84%和56%,以及80%和68%对79%和52%。14例患者(21%)复发(10例儿童和4例成人),中位复发时间分别为11个月和23个月,中位随访期分别为8个月和12个月;神经轴是最常见的复发部位(11例患者)。90%(9/10)的儿童复发属于高危组(8例未接受化疗)且发生在2年内;同样,所有成年复发均属于高危组;3例复发发生在2年后。单因素分析中,T分期、M分期、手术范围、脑脊液播散和风险类别是儿童年龄组无病生存的重要预后因素。5年时,T2、M0、近全切除、脑脊液播散阴性和低风险患者的无病生存率估计分别为87%、87%、78%、85%和85%,而T4、M+、仅活检、脑脊液播散阳性患者无一例达到5年无复发,高危患者中只有41%达到5年无复发(p=0.001、<0.001、<0.001、<0.001、<0.001)。未接受化疗的患者5年无病生存率为69%对76%(p=0.286)。对于成年患者;只有风险类别是一个重要的预后因素,低风险和高风险患者的5年无病生存率分别为100%对40%(p=0.03)。多因素分析中,只有风险类别和T分期是儿童年龄组无病生存的重要预后因素(p=0.042和0.

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