Picariello Stefania, Spennato Pietro, Roth Jonathan, Shimony Nir, Marini Alessandra, De Martino Lucia, Nicosia Giancarlo, Mirone Giuseppe, De Santi Maria Serena, Savoia Fabio, Errico Maria Elena, Quaglietta Lucia, Costantini Shlomi, Cinalli Giuseppe
Neuro-Oncology Unit, Department of Paediatric Oncology, Santobono-Pausilipon Children's Hospital, Via Mario Fiore 6, 80129 Naples, Italy.
Department of Women, Child and General and Specialized Surgery, University of Campania "Luigi Vanvitelli", Via Luigi De Crecchio 4, 80138 Naples, Italy.
Diagnostics (Basel). 2022 Mar 4;12(3):635. doi: 10.3390/diagnostics12030635.
Posterior fossa tumours (PFTs) in infants are very rare, and information on these tumours is scarce in the literature. This retrospective study reports their pathological characteristics and describes surgical aspects and treatment outcomes. A two-centre cohort of infants with PFTs treated from 2007 to 2018 was retrospectively reviewed. Patient characteristics, clinical, and treatment data were reviewed. Survival curves for progression-free survival (PFS) and overall survival (OS) were generated. Thirty-three infants were retrieved. There were 11 low grade and 22 high-grade tumours. The most common presenting symptom was intracranial hypertension. Fifteen children out of thirty-three progressed. Five-year PFS was significantly lower in children with high-grade tumours (38.3%) than those with low-grade tumours (69.3%), = 0.030. High-grade pathology was the only predictor of progression (HR 3.7, 95% CI 1.1-13.31), = 0.045. Fourteen children with high-grade tumours died, with a 5-year OS of 55.25%. PFTs in children below one year of age still represent a unique challenge. Infants with high-grade tumours display the worst outcomes and the lowest survival, indicating that more effective strategies are needed.
婴儿后颅窝肿瘤(PFTs)非常罕见,文献中关于这些肿瘤的信息也很少。这项回顾性研究报告了它们的病理特征,并描述了手术方面和治疗结果。对2007年至2018年在两个中心接受治疗的患有PFTs的婴儿队列进行了回顾性分析。回顾了患者特征、临床和治疗数据。生成了无进展生存期(PFS)和总生存期(OS)的生存曲线。共检索到33例婴儿。其中低级别肿瘤11例,高级别肿瘤22例。最常见的症状是颅内高压。33名儿童中有15名病情进展。高级别肿瘤患儿的5年PFS(38.3%)显著低于低级别肿瘤患儿(69.3%),P = 0.030。高级别病理是唯一的病情进展预测因素(HR 3.7,95%CI 1.1 - 13.31),P = 0.045。14名高级别肿瘤患儿死亡,5年OS为55.25%。一岁以下儿童的PFTs仍然是一个独特的挑战。高级别肿瘤婴儿的预后最差,生存率最低,这表明需要更有效的治疗策略。