关键词: Ependymoma Infratentorial neoplasms Medulloblastoma Pilocytic astrocytoma

Mesh : Humans Infratentorial Neoplasms / surgery Child Male Female Retrospective Studies Child, Preschool Neurosurgical Procedures / methods Adolescent Infant Treatment Outcome Latin America / epidemiology Survival Rate

来  源:   DOI:10.1007/s00381-024-06539-w

Abstract:
OBJECTIVE: We aim to report the epidemiology, surgical outcomes, and survival rates of pediatric patients with posterior fossa tumors in a large single-center case series.
METHODS: A retrospective analysis was conducted on pediatric patients who underwent surgical treatment for posterior fossa tumors between January 2011 and January 2019.
RESULTS: A total of 135 pediatric patients, with an average age of 7.5 years at diagnosis and a mean follow-up of 35.7 months, were included in the study. Most tumors were located within the midline, with ventriculomegaly observed in 71.4% of the patients. Pilocytic astrocytomas encompassed the majority of tumors (34.1%), followed by medulloblastomas (27.4%) and ependymomas (11.8%). Gross total resection (GTR) was achieved in 71.8% of the patients, with a recurrence rate of 20%. Surgical complications were observed in 25.9% of the patients. GTR significantly impacted 5-year overall survival (OS) and 4-year progression-free survival (PFS) in patients with posterior fossa tumors. Patients who underwent GTR had a 5-year OS of 89.7%, compared to 72.7% for near-total resection and 70.8% for subtotal resection. The 4-year PFS for patients who underwent GTR was 82.5%, whereas it was 63.6% for patients who underwent near-total resection and 54.2% for patients who underwent subtotal resection.
CONCLUSIONS: Surgical resection remains the main treatment for pediatric posterior fossa tumors, and higher resection rates are linked to better survival outcomes. Despite limited resources for molecular diagnosis, our institution has demonstrated that a specialized neurooncological center with a high surgical volume can still achieve favorable survival outcomes for these patients.
摘要:
目的:我们的目的是报告流行病学,手术结果,在大型单中心病例系列中,后颅窝肿瘤患儿的生存率。
方法:对2011年1月至2019年1月接受后颅窝肿瘤手术治疗的儿童患者进行回顾性分析。
结果:共有135名儿科患者,诊断时平均年龄为7.5岁,平均随访时间为35.7个月,包括在研究中。大多数肿瘤位于中线内,在71.4%的患者中观察到脑室增宽。毛细胞星形细胞瘤涵盖了大多数肿瘤(34.1%),其次是髓母细胞瘤(27.4%)和室管膜瘤(11.8%)。71.8%的患者实现了总切除(GTR),复发率为20%。25.9%的患者出现手术并发症。GTR显著影响后颅窝肿瘤患者的5年总生存期(OS)和4年无进展生存期(PFS)。接受GTR的患者5年OS为89.7%,与接近全切除的72.7%和次全切除的70.8%相比。接受GTR的患者的4年PFS为82.5%,而接受近全切除术的患者为63.6%,接受次全切除术的患者为54.2%.
结论:手术切除仍是小儿后颅窝肿瘤的主要治疗方法,更高的切除率与更好的生存结果相关。尽管分子诊断资源有限,我们的机构已经证明,对于这些患者,具有高手术量的专门神经肿瘤中心仍然可以取得良好的生存结局.
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