CNS germinoma: Disease control and long-term functional outcome for 12 children treated with craniospinal irradiation

CNS germinoma: Disease control and long-term functional outcome for 12 children treated with craniospinal irradiation
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DOI:
10.1016/s0360-3016(99)00375-2
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发表时间:
2000-03-15
影响因子:
7
通讯作者:
Kun, LE
Kun, LE
中科院分区:
医学1区
文献类型:
--
作者:
Merchant, TE;Sherwood, SH;Kun, LE

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目的:提供证据表明,单独的放射治疗形式的颅脊髓照射(CSI)和加强对原发部位的疾病提供了有效的疾病控制和有限的额外发病率的CNS生殖细胞瘤患者。方法和材料:12名中位年龄为12岁的患者,(范围9-16西尔斯)与中枢神经系统生殖细胞瘤的治疗与CSI(中位数25.6戈伊,范围23.4-32戈伊),并加强到原发部位的疾病(50.4戈伊,范围45-54戈伊),所有患者在放射治疗前均进行了活检,无患者接受化疗,结果:所有12例患者均存活,随访时间14-143个月,中位随访时间69个月。6/6例鞍上肿瘤和1/6例松果体肿瘤存在照射前内分泌缺陷;随访时,年龄和性别调整后的照射前和照射后身高和体重之间没有实质性差异。与长期随访,有没有显着差异pre-and postirradiation全面,口头,和性能IQ scores.Conclusions:这项研究证实了单独的放射治疗的能力,以实现疾病控制与儿科患者的成功率高,并表明,这些患者面临的治疗毒性可能低于预期。由于这些患者在诊断时存在大量的既存疾病,并且可能处于辐射相关副作用的可能性相对较小的年龄,因此可能难以证明治疗替代方案的优效性。(C)2000 Elsevier Science Inc.
Purpose: To provide evidence that radiation therapy alone in the form of craniospinal irradiation (CSI) and a boost to the primary site of disease provides effective disease control and limited additional morbidity for patients with CNS germinoma.Methods and Materials: Twelve patients with a median age of It rears (range 9-16 Sears) with CNS germinoma were treated with CSI (median 25.6 Gy, range 23.4-32 Gy) and a boost to the primary site of disease (50.4 Gy, range 45-54 Gy) between January 1987 and June 1998, All patients were biopsied prior to radiation therapy and none received chemotherapy, No patients were lost to follow-up and the majority had long-term (> 45 month) pre- and postirradiation endocrine and psychology assessment.Results: All 12 patients are alive and no failures have occurred with a median follow-up of 69 months (range 14-143 months). Preirradiation endocrine deficiencies were present in 6 of 6 suprasellar tumors and 1 of 6 pineal tumors; with follow-up there was no substantial difference between age and gender adjusted pre- and postirradiation stature and weight. With long-term follow-up, there were no significant differences between pre- and postirradiation full-scale, verbal, and performance IQ scores.Conclusions: This study confirms the ability of radiation therapy alone to achieve disease control with a high rate of success in pediatric patients and demonstrates that the treatment toxicity faced by these patients may be less than anticipated. Because these patients present with substantial preexisting morbidity at diagnosis and may be of an age where the potential for radiation-related side effects is relatively small, the superiority of treatment alternatives may be difficult to prove. (C) 2000 Elsevier Science Inc.