Rajeshwari Madhu, Sharma Mehar Chand, Kakkar Aanchal, Nambirajan Aruna, Suri Vaishali, Sarkar Chitra, Singh Manmohan, Saran Ravindra Kumar, Gupta Rakesh Kumar
Department of Pathology, All India Institute of Medical Sciences, New Delhi, 110029, India.
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, 110029, India.
J Neurooncol. 2016 Apr;127(2):271-8. doi: 10.1007/s11060-015-2047-z. Epub 2016 Jan 2.
Ependymomas are relatively uncommon gliomas with poor prognosis despite recent advances in neurooncology. Molecular pathogenesis of ependymomas is not extensively studied. Lack of correlation of histological grade with patient outcome has directed attention towards identification of molecular alterations as novel prognostic markers. Recently, 1q gain has emerged as a potential prognostic marker, associated with decreased survival, especially in posterior fossa, high grade tumors. Cases of intracranial ependymomas were retrieved. Tumors were graded using objective criteria to supplement WHO grading. Fluorescence in situ hybridization for 1q gain was performed on formalin-fixed paraffin embedded sections. Eighty-one intracranial ependymomas were analyzed. Pediatric (76%) and infratentorial (70%) ependymomas constituted the majority. 1q gain was seen in 27 cases (33%), was equally frequent in children (34%) and adults (32%), supratentorial (37%) and infratentorial (32%) location, grade II (33%) and III (25%) tumors. Recurrence was noted in 24 cases and death in 7 cases with 5-year progression-free and overall-survival rates of 37% and 80%, respectively. Grade II tumors had a better survival than grade III tumors; histopathological grade was the only prognostically significant marker. 1q gain had no prognostic significance. 1q gain is frequent in ependymomas in Indian patients, seen across all ages, sites and grades, and thus is likely an early event in pathogenesis. The prognostic value of 1q gain, remains uncertain, and multicentric pooling of data is required. A histopathological grading system using objective criteria correlates well with patient outcome and can serve as an economical option for prognostication of ependymomas.
室管膜瘤是相对罕见的胶质瘤，尽管神经肿瘤学最近取得了进展，但其预后较差。室管膜瘤的分子发病机制尚未得到广泛研究。组织学分级与患者预后缺乏相关性，促使人们将注意力转向识别分子改变作为新的预后标志物。最近，1q增益已成为一种潜在的预后标志物，与生存率降低相关，尤其是在后颅窝高级别肿瘤中。检索了颅内室管膜瘤病例。使用客观标准对肿瘤进行分级以补充世界卫生组织（WHO）分级。在福尔马林固定石蜡包埋切片上进行1q增益的荧光原位杂交。分析了81例颅内室管膜瘤。小儿室管膜瘤（76%）和幕下室管膜瘤（70%）占大多数。27例（33%）出现1q增益，在儿童（34%）和成人（32%）、幕上（37%）和幕下（32%）部位、II级（33%）和III级（25%）肿瘤中发生率相同。24例出现复发，7例死亡，5年无进展生存率和总生存率分别为37%和80%。II级肿瘤的生存率高于III级肿瘤；组织病理学分级是唯一具有预后意义的标志物。1q增益无预后意义。1q增益在印度患者的室管膜瘤中很常见，在所有年龄、部位和分级中均可见，因此可能是发病机制中的早期事件。1q增益的预后价值仍不确定，需要多中心数据汇总。使用客观标准的组织病理学分级系统与患者预后密切相关，可作为室管膜瘤预后评估的经济选择。
