Adult medulloblastoma: Prognostic factors and patterns of relapse
Adult medulloblastoma: Prognostic factors and patterns of relapse
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DOI：
10.1097/00006123-200009000-00018
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发表时间：
2000-09-01
期刊：
影响因子：
4.8
通讯作者：
Loeffler, JS
中科院分区：
文献类型：
--
作者：
Chan, AW;Tarbell, NJ;Loeffler, JS
目的：探讨磁共振成像时代成人髓母细胞瘤的复发模式及预后因素。方法：在1986年至1996年期间，在我们的机构治疗了32例局限于颅脊髓轴的成神经管细胞瘤的成年患者（年龄大于或等于16岁）。20例为典型组织学特征，12例为结缔组织变异体。Chang分期分布如下：T1, 2；T2, 17;T3、10;T4, 3;M0, 24;M1, 1;平方米,4;M3, 3。9例患者出现脑干侵犯。病变中线13例，外侧19例。完全切除17例，次全切除6例，部分切除5例，活检4例。所有患者术后均接受放射治疗，中位剂量为全颅脊髓轴36 Cy，后窝55 Cy。化疗24例（放疗前20例，放疗后3例，放疗前后各1例）。结果：中位随访时间为5.4年，17例患者复发。5年和8年的总生存率分别为83%和45%，无病生存率分别为57%和40%。5年和8年后窝控制率分别为67%和59%。29%的复发发生在治疗后5年以上。后窝是最常见的复发部位。在单因素分析中，影响后窝控制的不利因素包括完全切除（P < 0.001）、脑干侵犯（P = 0.02）和化疗（P = 0.03）。总放疗时间在预测后颅窝控制方面具有微弱的统计学意义，治疗时间小于48天和大于48天的5年后颅窝控制率分别为81%和49% （P = 0.06）。在一项多因素分析中，完全切除可以改善后窝控制（P = 0.02）和无病生存率（P = 0.02）。在8名单独接受放疗的低风险患者中，有3名复发部位为骨。结论：成神经管细胞瘤晚期复发是常见的，长期随访监测是重要的。由于单纯放疗的低危患者发生全身衰竭的风险较高，化疗对这组患者的作用有待进一步研究。完全切除，脑干无侵犯，总放疗时间少于48天是重要的预后因素。
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, greater than or equal to 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 Cy to the entire craniospinal axis and 55 Cy 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.