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儿童高级别星形细胞瘤:影像学上的完全切除与良好的长期预后相关。

High-grade astrocytomas in children: radiologically complete resection is associated with an excellent long-term prognosis.

作者信息

Campbell J W, Pollack I F, Martinez A J, Shultz B

机构信息

Department of Neurosurgery, University of Pittsburgh School of Medicine, Pennsylvania, USA.

出版信息

Neurosurgery. 1996 Feb;38(2):258-64. doi: 10.1097/00006123-199602000-00006.

DOI:10.1097/00006123-199602000-00006
PMID:8869052
Abstract

High-grade astrocytomas comprise approximately 10% of intracranial tumors in children. A better prognosis in children than in adults has been reported for patients with these neoplasms, although the reasons for this survival advantage are uncertain. To determine whether any consistent factors were associated with long-term survival, we reviewed the records of 31 children with high-grade non-brain stem gliomas who were treated at our institution between 1975 and 1992. Histology was reviewed and classified according to the World Health Organization scheme, and neuroimaging studies were examined to determine the extent of resection, in both instances by individuals who were unaware of the patients' outcomes. The median overall survival for the 28 patients who survived the perioperative period was 18.5 months; 10 (36%) are currently alive, with a median follow-up of 70.5 months. The median progression-free survival (PFS) was 10.5 months; eight patients (29%) remain progression-free with a median follow-up of 78 months. The extent of resection at initial operation was associated most closely with PFS and overall survival as revealed by multivariate analysis. The 14 patients who underwent subtotal (< 90%) resection and the 7 who underwent near-total (90-99%) resection had median PFS of 5.5 and 11 months, respectively (P = 0.38), and overall survival of 10.5 and 25 months, respectively (P = 0.02). None of the seven patients who underwent gross total removal of tumor as confirmed by postoperative imaging had disease progression, with a median follow-up of 84 months (P < 0.0001). All of the tumors that underwent gross total resection were situated within the cerebral hemispheres; five of the seven patients had seizures as a presenting symptom. Both tumor location and seizures were significantly associated with outcome as determined by univariate analysis, but because of the overwhelming impact of resection extent on outcome, these factors were not independently associated with outcome as revealed by multivariate analysis. Histology was associated with outcome in the subgroup of patients with incompletely resected hemispheric tumors, in which children with anaplastic astrocytoma had a significantly better PFS than those with glioblastoma multiforme (P = 0.009). In summary, our results support the role of cytoreductive surgery in the treatment of cerebral hemispheric high-grade astrocytomas in children, which may encompass a biologically distinct group of tumors that, by virtue of their location and growth characteristics, are amenable to aggressive resection. The prognosis for children with deep-seated lesions and for those with subtotally resected hemispheric lesions is generally poor with conventional therapy.

摘要

高级别星形细胞瘤约占儿童颅内肿瘤的10%。据报道,这些肿瘤患儿的预后比成人好,尽管这种生存优势的原因尚不清楚。为了确定是否有任何一致的因素与长期生存相关,我们回顾了1975年至1992年期间在我们机构接受治疗的31例高级别非脑干胶质瘤患儿的记录。组织学根据世界卫生组织的方案进行回顾和分类,神经影像学研究用于确定切除范围,这两项工作均由不了解患者预后的人员进行。28例围手术期存活患儿的中位总生存期为18.5个月;10例(36%)目前仍存活,中位随访时间为70.5个月。中位无进展生存期(PFS)为10.5个月;8例患者(29%)仍无进展,中位随访时间为78个月。多因素分析显示,初次手术时的切除范围与PFS和总生存期最密切相关。14例行次全切除(<90%)的患者和7例行近全切除(90-99%)的患者,中位PFS分别为5.5个月和11个月(P = 0.38),总生存期分别为10.5个月和25个月(P = 0.02)。术后影像学证实7例肿瘤全切的患者均无疾病进展,中位随访时间为84个月(P < 0.0001)。所有接受肿瘤全切的肿瘤均位于大脑半球;7例患者中有5例以癫痫为首发症状。单因素分析确定肿瘤位置和癫痫均与预后显著相关,但由于切除范围对预后的压倒性影响,多因素分析显示这些因素与预后无独立相关性。在半球肿瘤未完全切除的患者亚组中,组织学与预后相关,其中间变性星形细胞瘤患儿的PFS明显优于多形性胶质母细胞瘤患儿(P = 0.009)。总之,我们的结果支持了减瘤手术在儿童大脑半球高级别星形细胞瘤治疗中的作用,这些肿瘤可能构成一个生物学上独特的肿瘤组,由于其位置和生长特征,适合进行积极切除。对于深部病变患儿和半球病变次全切除的患儿,传统治疗的预后通常较差。

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