Department of Experimental Medicine, Sapienza University of Rome, Viale Regina Elena 324, 00161, Rome, Italy.
Neurosurgery Unit, Department of Neuroscience and Neurorehabilitation, IRCCS Bambino Gesù Children's Hospital, Rome, Italy.
Childs Nerv Syst. 2021 Mar;37(3):771-778. doi: 10.1007/s00381-020-04559-w. Epub 2020 Mar 11.
Pediatric low-grade gliomas (pLGGs), the most frequent pediatric brain tumors, include different entities harboring distinct histological and molecular features. A major limitation in the development of treatments for these tumors is the absence of reliable in vitro models that would allow a better understanding of the molecular mechanisms that support their growth. Surgical excision is the primary treatment method and the extent of resection represents one of the strongest prognostic factors. pLGGs that cannot be completely resected are prone to recur and associated with relapses and extensive morbidities, thus remaining a major clinical challenge.
We established a protocol to successfully derive primary patient-derived pLGG cells and to fully characterize them from a molecular point of view.
Primary patients-derived pLGG cells were extensively analyzed in order to confirm their reliability as cellular models. Specifically, we evaluated the growth rate, senescence, and molecular features, such as BRAF mutational status, methylation, and protein expression profile.
This study extensively describes pLGG primary cellular models in terms of isolation, culture method, and molecular characterization that can be used to investigate pLGG biology.
小儿低级别胶质瘤（pLGGs）是最常见的小儿脑肿瘤，包括具有不同组织学和分子特征的不同实体。开发这些肿瘤治疗方法的主要限制是缺乏可靠的体外模型，无法更好地了解支持其生长的分子机制。手术切除是主要的治疗方法，切除范围是最强的预后因素之一。无法完全切除的 pLGG 容易复发，并伴有复发和广泛的发病率，因此仍然是一个主要的临床挑战。
我们建立了一个方案，成功地从患者中分离出原发性 pLGG 细胞，并从分子角度对其进行了全面的表征。
为了确认其作为细胞模型的可靠性，对原发性患者衍生的 pLGG 细胞进行了广泛分析。具体来说，我们评估了生长速度、衰老和分子特征，如 BRAF 突变状态、甲基化和蛋白表达谱。
本研究从分离、培养方法和分子特征等方面对 pLGG 原代细胞模型进行了广泛描述，可用于研究 pLGG 生物学。
