Unidad de Neurocirugía Pediátrica, Hospital Universitario y Politécnico La Fe, Avda Fernando Abril Martorell, 106, 46026, Valencia, Spain.
Childs Nerv Syst. 2024 Feb;40(2):407-416. doi: 10.1007/s00381-023-06194-7. Epub 2023 Oct 27.
To review a multicentric series of lateral-type posterior fossa ependymomas operated in the last ten years and to analyze the factors related to clinical evolution and tumor survival.
Descriptive, retrospective study. Active members of the Spanish Society of Pediatric Neurosurgery were invited to participate in this multicentric study. Clinical and radiological data were incorporated to an open database. The role of histologic grade, grade of resection, postoperative morbidities, and clinical follow-up was evaluated through bivariate associations (chi-square), Kaplan-Meier's curves (log-rank test), and multivariate analysis (binary logistic regression).
Fourteen centers entered the study, and 25 cases with a minimum follow-up of 6 months were included. There were 13 boys and 12 girls with a mean age close to 3 years. Mean tumor volume at diagnosis was over 60 cc. A complete resection was achieved in 8 patients and a near-total resection in 5 cases. Fifteen tumors were diagnosed as ependymoma grade 2 and ten as ependymoma grade 3. Major morbidity occurred postoperatively in 14 patients but was resolved in twelve within 6 months. There were six cases of death and 11 cases of tumor progression along the observation period. Mean follow-up was 44.8 months. Major morbidity was significantly associated with histologic grade but not with the degree of resection. Overall and progression-free survival were significantly associated with complete surgical resection. At the last follow-up, 16 patients carried a normal life, and three displayed a mild restriction according to Lansky's scale.
Lateral-type posterior fossa ependymomas constitute a specific pathologic and clinical tumor subtype with bad prognosis. Gross total resection is the goal of surgical treatment, for it significantly improves prognosis with no additional morbidity. Neurological deficits associated to lower cranial nerve dysfunction are common, but most are transient. Deeper genetic characterization of these tumors may identify risk factors that guide new treatments and stratification of adjuvant therapies.
回顾过去十年间在多个中心进行的外侧型后颅窝室管膜瘤手术，并分析与临床转归和肿瘤生存相关的因素。
这是一项描述性、回顾性研究。邀请西班牙小儿神经外科学会的活跃成员参与这项多中心研究。将临床和影像学数据纳入一个开放数据库。通过双变量关联（卡方检验）、Kaplan-Meier 曲线（对数秩检验）和多变量分析（二项逻辑回归）评估组织学分级、切除程度、术后并发症和临床随访的作用。
共有 14 个中心参与了这项研究，共纳入了 25 例至少随访 6 个月的患者。其中有 13 名男孩和 12 名女孩，平均年龄接近 3 岁。诊断时肿瘤体积平均超过 60cc。8 例患者实现了完全切除，5 例患者实现了近全切除。15 例肿瘤被诊断为 2 级室管膜瘤，10 例为 3 级室管膜瘤。14 例患者术后出现严重并发症，但 12 例在 6 个月内得到解决。在观察期间，有 6 例死亡，11 例肿瘤进展。平均随访时间为 44.8 个月。主要并发症与组织学分级显著相关，但与切除程度无关。总体生存率和无进展生存率与完全手术切除显著相关。在最后一次随访时，16 名患者过着正常的生活，3 名患者根据 Lansky 量表显示轻度受限。
外侧型后颅窝室管膜瘤是一种具有不良预后的特定病理和临床肿瘤亚型。肿瘤全切除是手术治疗的目标，因为它显著改善了预后，且不会增加并发症。与颅神经功能障碍相关的神经功能缺损很常见，但大多是暂时的。对这些肿瘤进行更深入的遗传特征分析可能会确定风险因素，从而指导新的治疗方法和辅助治疗的分层。
