Department of Pediatric and Adolescent Oncology, Gustave Roussy Cancer Institute, Villejuif, France.
Curr Opin Oncol. 2011 Nov;23(6):638-42. doi: 10.1097/CCO.0b013e32834b5310.
Ependymomas remain a therapeutic challenge in pediatric neuro-oncology. These tumors are chemoresistant and rather radioresistant and until recently little was known about their biology.
Histopathological grading of ependymomas according to the WHO classification is neither reproducible, nor correlated with outcome, especially in young children. Characterization of molecular abnormalities in ependymomas offers now a better understanding of their initiation and progression; different biological subtypes of tumors have been described and would need further validation. The identification of new prognostic biomarkers, such as tenascin-C overexpression or chromosome 1q gain, will considerably help patient stratification in future trials. Finally, the recent discovery of specific pathways involved in ependymomas oncogenesis, such as Notch-1or EPHB2 offers new perspectives for the development of targeted therapies.
A comprehensive biological work-out including CGHarray and immunohistochemistry for specific biomarkers should now be recommended for the current management of pediatric ependymoma, especially in young children if radiotherapy has to be omitted in the first line of treatment.
室管膜瘤仍然是儿童神经肿瘤学治疗的一个挑战。这些肿瘤对化疗耐药，放疗也相对抗拒，直到最近，人们对其生物学特性还知之甚少。
根据世界卫生组织分类对室管膜瘤进行组织病理学分级既不可重复，也与预后无关，尤其是在幼儿中。室管膜瘤中分子异常的特征现在提供了对其发生和进展的更好理解；已经描述了不同的肿瘤生物学亚型，需要进一步验证。鉴定新的预后生物标志物，如 tenascin-C 过表达或 1q 染色体获得，将极大地帮助未来试验中的患者分层。最后，最近发现 Notch-1 或 EPHB2 等参与室管膜瘤发生的特定途径为靶向治疗的发展提供了新的前景。
包括 CGH 芯片和特定生物标志物的免疫组化在内的全面生物学研究现在应该被推荐用于当前儿童室管膜瘤的治疗，特别是在幼儿中，如果一线治疗必须省略放疗。
