Gajjar A, Sanford R A, Heideman R, Jenkins J J, Walter A, Li Y, Langston J W, Muhlbauer M, Boyett J M, Kun L E
St Jude Children's Research Hospital/Le Bonheur Children's Medical Center Brain Tumor Team, Memphis, TN 38105-2794, USA.
J Clin Oncol. 1997 Aug;15(8):2792-9. doi: 10.1200/JCO.1997.15.8.2792.
To evaluate the impact of primary tumor site, age at diagnosis, extent of resection, and histology on progression-free survival (PFS) in pediatric low-grade astrocytoma.
Medical, pathologic, and imaging information were reviewed for 142 children (ages 2 months to 19 years) with low-grade astrocytoma treated between January 1984 and July 1994. Gross total resection (GTR) was attempted for cerebellar and cerebral hemisphere tumors, with biopsy or less aggressive resection used predominantly for tumors in other sites. Surgery was followed by observation in 107 cases, radiation therapy in 31, and chemotherapy in four.
The overall survival rate was 90% +/- 3% (SE) at 4 years. PFS was significantly better for patients with cerebellar and cerebral hemisphere tumors (n = 75) than those with tumors in all other sites (P = .0006). Within the former group, there was no significant difference in PFS for patients in whom GTR was achieved versus those with incomplete resections (4-year estimates, 89% and 77%, respectively). Histology (juvenile pilocytic v astrocytoma not otherwise specified [NOS]) was not related to PFS in an analysis that controlled for tumor site and patient age. Patients younger than 5 years at diagnosis had a significantly poorer PFS than older children, regardless of histology (P < .03) or tumor site (P < .002). Treatment for progressive/recurrent disease was effective in a majority of patients, but appeared more successful in patients with hemispheric than thalamic or hypothalamic tumors.
The overall survival in this series of pediatric low-grade astrocytomas is excellent. Age at diagnosis and tumor location, but not histology, had a significant impact on PFS. Efforts to improve treatment outcome should focus on young patients (< 5 years) and on those with central midline tumors. The majority of patients with completely resected hemispheric tumors were monitored without further therapy, which supports attempted GTR of cerebral and cerebellar hemisphere low-grade astrocytoma.
评估原发肿瘤部位、诊断时年龄、切除范围和组织学对小儿低级别星形细胞瘤无进展生存期（PFS）的影响。
回顾了1984年1月至1994年7月期间接受治疗的142例（年龄2个月至19岁）低级别星形细胞瘤患儿的医学、病理和影像学信息。对于小脑和大脑半球肿瘤尝试进行全切除（GTR），其他部位肿瘤主要采用活检或不太积极的切除方式。107例患者术后进行观察，31例接受放射治疗，4例接受化疗。
4年总生存率为90%±3%（SE）。小脑和大脑半球肿瘤患者（n = 75）的PFS明显优于所有其他部位肿瘤患者（P = 0.0006）。在前一组中，实现GTR的患者与切除不完全的患者的PFS无显著差异（4年估计值分别为89%和77%）。在控制肿瘤部位和患者年龄的分析中，组织学（青少年毛细胞型星形细胞瘤，未另作说明[NOS]）与PFS无关。诊断时年龄小于5岁的患者，无论组织学（P < 0.03）或肿瘤部位（P < 0.002）如何，其PFS均明显低于年龄较大的儿童。对进展性/复发性疾病的治疗在大多数患者中有效，但在半球肿瘤患者中似乎比丘脑或下丘脑肿瘤患者更成功。
这一系列小儿低级别星形细胞瘤的总体生存率良好。诊断时年龄和肿瘤位置而非组织学对PFS有显著影响。改善治疗结果的努力应集中在年轻患者（<5岁）和中枢中线肿瘤患者身上。大多数半球肿瘤完全切除的患者无需进一步治疗即可进行监测，这支持对大脑和小脑半球低级别星形细胞瘤尝试进行GTR。
