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伴有脑干受累的髓母细胞瘤:全切除对预后的影响。

Medulloblastoma with brain stem involvement: the impact of gross total resection on outcome.

作者信息

Gajjar A, Sanford R A, Bhargava R, Heideman R, Walter A, Li Y, Langston J W, Jenkins J J, Muhlbauer M, Boyett J, Kun L E

机构信息

Le Bonheur Children's Medical Center, Brain Tumor Team, Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, TN 38101-0318, USA.

出版信息

Pediatr Neurosurg. 1996 Oct;25(4):182-7. doi: 10.1159/000121121.

Abstract

We studied the impact of gross total resection on progression-free survival (PFS) and postoperative morbidity in 40 children with locally advanced medulloblastoma characterized by tumor invading the brain stem. These patients represented 40% of children treated for newly diagnosed medulloblastoma at a pediatric oncology center over a 10-year period. All patients underwent aggressive initial surgical resection. Review of surgical and neuroimaging findings documented gross total resection in 13 cases, near-total resection (< 1.5 cm2 residual tumor on imaging) in 14 cases, and subtotal resection (> than 50% resection with > or = 1.5 cm2 residual) in 13 cases. Overall, 85% of patients had a > 90% resection. Subsequent therapy comprised craniospinal irradiation in all cases and chemotherapy on institutional or cooperative group protocols in 35 cases. At a median follow-up of 4 years, postirradiation PFS is 61% (SE = 10%). There was no difference in PFS for patients who underwent gross total resection compared to those with any detectable residual tumor (p > 0.70). The posterior fossa syndrome occurred in 25% of cases, and had no apparent relationship to the extent of resection (p > 0.5, exact test). In this series, true gross total resection was not associated with a PFS advantage when compared to strictly defined near-total and subtotal resection. Although there was no operative mortality, the frequency of the posterior fossa syndrome is of concern and emphasizes the need for careful consideration of the risk/benefit ratio in the surgical approach to this subgroup of patients.

摘要

我们研究了全切除对40例局部晚期髓母细胞瘤患儿无进展生存期(PFS)和术后发病率的影响,这些患儿的肿瘤侵犯脑干。在10年期间,这些患者占小儿肿瘤中心新诊断髓母细胞瘤患儿的40%。所有患者均接受了积极的初始手术切除。回顾手术和神经影像学检查结果显示,13例实现了全切除,14例为近全切除(影像学上残留肿瘤<1.5 cm²),13例为次全切除(切除>50%且残留≥1.5 cm²)。总体而言,85%的患者切除率>90%。后续治疗包括所有病例均进行颅脊髓照射,35例根据机构或合作组方案进行化疗。中位随访4年时,放疗后PFS为61%(标准误=10%)。与有任何可检测残留肿瘤的患者相比,接受全切除的患者PFS无差异(p>0.70)。25%的病例发生了后颅窝综合征,与切除范围无明显关系(p>0.5,精确检验)。在本系列中,与严格定义的近全切除和次全切除相比,真正的全切除并未带来PFS优势。虽然没有手术死亡,但后颅窝综合征的发生率令人担忧,强调在对该亚组患者的手术方法中需要仔细考虑风险/获益比。

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