Department of Pediatrics and Pediatric Hematology/Oncology, University Children's Hospital, Carl von Ossietzky University of Oldenburg, Klinikum Oldenburg AöR, 26133, Oldenburg, Germany.
Division of Epidemiology and Biometry, Carl von Ossietzky University of Oldenburg, 26129, Oldenburg, Germany.
J Neurooncol. 2023 Jul;163(3):555-563. doi: 10.1007/s11060-023-04344-0. Epub 2023 Jul 4.
Pediatric meningioma differs not only in its rare incidence from the adult meningioma, but also in its clinical characteristics. Many treatment approaches of pediatric meningioma are based on the study results of adult meningioma studies. The aim of this study was to explore the clinical and epidemiological characteristics of pediatric meningioma.
Data on pediatric patients diagnosed between 1982 and 2021 with NF2-associated or sporadic meningioma and recruited in the trials/registries HIT-ENDO, KRANIOPHARYNGEOM 2000/2007 and KRANIOPHARYNGEOM Registry 2019 were retrospectively analyzed for clinical characteristics, etiology, histology, therapy, and outcome.
One hundred fifteen study participants were diagnosed with sporadic or NF2-associated meningioma at a median age of 10.6 years. There was a 1:1 sex ratio, with 14% of study participants suffering from NF2. 46% of the meningiomas were located hemispherically, 17% at the optic nerve/ intraorbital and 10% ventricularly. Multiple meningiomas were detected in 69% of NF2 patients and in 9% of sporadic meningiomas. 50% of the meningiomas were WHO grade I, 37% WHO grade II and 6% WHO grade III. Progressions or recurrences occurred after a median interval of 1.9 years. Eight patients (7%) died, 3 of them due to disease. The event-free survival was higher for WHO grade I than for WHO grade II meningioma patients (p = 0.008).
The major difference to the preceding literature could be found in the distribution of different WHO grades and their influence on event-free survival. Prospective studies are warranted to assess the impact of different therapeutic regimens.
NCT00258453; NCT01272622; NCT04158284.
小儿脑膜瘤不仅在发病率上与成人脑膜瘤不同，而且在临床特征上也有所不同。许多小儿脑膜瘤的治疗方法都是基于成人脑膜瘤研究的研究结果。本研究旨在探讨小儿脑膜瘤的临床和流行病学特征。
回顾性分析了 1982 年至 2021 年间在 HIT-ENDO、KRANIOPHARYNGEOM 2000/2007 和 KRANIOPHARYNGEOM 登记处 2019 试验/登记处中诊断为 NF2 相关或散发性脑膜瘤的患儿患者的临床特征、病因、组织学、治疗和结局。
115 名研究参与者被诊断为散发性或 NF2 相关脑膜瘤，中位年龄为 10.6 岁。男女比例为 1:1，有 14%的研究参与者患有 NF2。46%的脑膜瘤位于半球，17%位于视神经/眶内，10%位于脑室。69%的 NF2 患者和 9%的散发性脑膜瘤患者发现多发性脑膜瘤。50%的脑膜瘤为 WHO 分级 I，37%为 WHO 分级 II，6%为 WHO 分级 III。中位间隔 1.9 年后出现进展或复发。8 名患者（7%）死亡，其中 3 名死于疾病。WHO 分级 I 患者的无事件生存时间高于 WHO 分级 II 患者（p=0.008）。
与之前的文献相比，主要的区别在于不同的 WHO 分级及其对无事件生存的影响。需要前瞻性研究来评估不同治疗方案的影响。
NCT00258453；NCT01272622；NCT04158284。
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