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成人髓母细胞瘤:预后因素及复发模式

Adult medulloblastoma: prognostic factors and patterns of relapse.

作者信息

Chan A W, Tarbell N J, Black P M, Louis D N, Frosch M P, Ancukiewicz M, Chapman P, Loeffler J S

机构信息

Department of Radiation Oncology, Massachusetts General Hospital, Boston 02114, USA.

出版信息

Neurosurgery. 2000 Sep;47(3):623-31; discussion 631-2. doi: 10.1097/00006123-200009000-00018.

Abstract

OBJECTIVE

To determine the patterns of relapse and the prognostic factors for adult medulloblastomas treated in the magnetic resonance imaging era.

METHODS

Between 1986 and 1996, 32 adult patients (age, > or =16 yr) with medulloblastomas confined to the craniospinal axis were treated in our institutions. Twenty cases involved classic histological features and 12 involved the desmoplastic variant. The Chang staging distribution was as follows: T1, 2; T2, 17; T3, 10; T4, 3; M0, 24; M1, 1; M2, 4; M3, 3. Brainstem invasion was present in nine patients. Lesions were midline in 13 cases and lateral in 19. Resection was complete in 17 cases, subtotal in 6, and partial in 5, with biopsy only in 4 cases. All patients received postoperative radiotherapy, with median doses of 36 Gy to the entire craniospinal axis and 55 Gy to the posterior fossa. Twenty-four patients received chemotherapy (20 before radiotherapy, 3 after radiotherapy, and 1 before and after radiotherapy).

RESULTS

With a median follow-up period of 5.4 years, 17 patients experienced recurrences. At 5 and 8 years, overall survival rates were 83 and 45% and disease-free survival rates were 57 and 40%, respectively. The 5- and 8-year posterior fossa control rates were 67 and 59%, respectively. Twenty-nine percent of all relapses occurred more than 5 years after treatment. The posterior fossa was the most common site of relapses. In univariate analyses, factors adversely affecting posterior fossa control were less than complete resection (P<0.001), the presence of brainstem invasion (P = 0.02), and the use of chemotherapy (P = 0.03). The overall radiotherapy duration was marginally significant in predicting posterior fossa control, with 5-year posterior fossa control rates of 81 and 49% for durations of less than 48 days and 48 days or more, respectively (P = 0.06). In a multivariate analysis, complete resection was predictive of improved posterior fossa control (P = 0.02) and disease-free survival (P = 0.02) rates. Of the eight low-risk patients who received radiotherapy alone, three experienced recurrences in the bone as the only site of relapse.

CONCLUSION

Late relapse is common among adult patients with medulloblastomas, and long-term follow-up monitoring is important. Because of the high risk of systemic failure among the low-risk patients treated with radiotherapy alone, the role of chemotherapy for this group of patients needs to be further investigated. Complete resection, the absence of brainstem invasion, and an overall radiotherapy duration of less than 48 days are important prognostic factors.

摘要

目的

确定在磁共振成像时代接受治疗的成人髓母细胞瘤的复发模式及预后因素。

方法

1986年至1996年间,我们机构治疗了32例年龄≥16岁、髓母细胞瘤局限于颅脊柱轴的成年患者。20例具有经典组织学特征,12例为促纤维增生型变异型。Chang分期分布如下:T1期2例;T2期17例;T3期10例;T4期3例;M0期24例;M1期1例;M2期4例;M3期3例。9例患者存在脑干侵犯。13例病变位于中线,19例位于外侧。17例患者实现完全切除,6例次全切除,5例部分切除,仅4例进行活检。所有患者均接受术后放疗,全颅脊柱轴的中位剂量为36 Gy,后颅窝为55 Gy。24例患者接受化疗(20例在放疗前,3例在放疗后,1例放疗前后均接受化疗)。

结果

中位随访期为5.4年,17例患者出现复发。5年和8年时,总生存率分别为83%和45%,无病生存率分别为57%和40%。5年和8年后颅窝控制率分别为67%和59%。所有复发中有29%发生在治疗后5年以上。后颅窝是最常见的复发部位。单因素分析中,对后颅窝控制产生不利影响的因素包括未完全切除(P<0.001)、存在脑干侵犯(P = 0.02)以及使用化疗(P = 0.03)。总体放疗疗程在预测后颅窝控制方面有一定意义,疗程少于48天和48天及以上的5年后颅窝控制率分别为81%和49%(P = 0.06)。多因素分析中,完全切除可预测后颅窝控制(P = 0.02)及无病生存率(P = 0.02)提高。在仅接受放疗的8例低风险患者中,3例在骨内复发,且骨是唯一的复发部位。

结论

成人髓母细胞瘤患者中晚期复发常见,长期随访监测很重要。由于仅接受放疗的低风险患者存在较高的全身衰竭风险,化疗在该组患者中的作用需要进一步研究。完全切除、无脑干侵犯以及总体放疗疗程少于48天是重要的预后因素。

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