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Central nervous system neuroepithelial tumors with MN1-alteration: an individual patient data meta-analysis of 73 cases.
Brain Tumor Pathology ( IF 3.3 ) Pub Date : 2020-06-29 , DOI: 10.1007/s10014-020-00372-0
Wanwan Chen 1, 2 , Yu Yang Soon 3 , Patricia Diana Pratiseyo 4 , Ratna Sutanto 5 , Lutfi Hendriansyah 6 , Chik Hong Kuick 2 , Kenneth T E Chang 2, 7 , Char Loo Tan 2, 8, 9
Affiliation  

MN1 alteration characterizes a recently described group of neuroepithelial tumors with varied morphological features. In cIMPACT-NOW update 6, only those with astroblastoma morphology has been accepted as a newly recognized tumor type, whereas the rest of morphological variants are considered lesions sub-judice. We perform an individual patient data meta-analysis of MN1-altered neuroepithelial tumors comprising a total of 73 cases, in order to study the survival data and predictive markers for better diagnosis and management of this rare molecular entity. The 5- and 10-year progression-free survival are 38% and 0%, whereas the 5- and 10-year overall survival are 89% and 55%, respectively. Among all the morphological variants of MN1-altered tumor, astroblastoma morphology is significantly associated with an improved overall survival, emphasizing the importance of providing an integrated histologic and molecular diagnosis. Histological grading within the molecularly-defined MN1-altered astroblastoma remains controversial. In tumors with astroblastoma morphology, the odds of MN1-altered status among patients less than 15-year-old is 10.5 times that of those 15-year-old and older, and female of 9.4 times that of the male gender. Gross tumor resection appears as main treatment modality for better disease control based on observational data.



中文翻译:

MN1改变的中枢神经系统上皮性肿瘤:73例患者的个人数据荟萃分析。

MN1改变的特点是最近描述的一组具有不同形态特征的神经上皮肿瘤。在cIMPACT-NOW更新6中,只有具有星形母细胞瘤形态的那些才被接受为新识别的肿瘤类型,而其余形态变异被认为是对病变的判断。为了研究生存数据和预测标记物,以更好地诊断和管理这种罕见的分子实体,我们对MN1改变的神经上皮性肿瘤进行了单独的患者数据荟萃分析,共计73例。5年和10年无进展生存率分别为38%和0%,而5年和10年总生存率分别为89%和55%。在MN1的所有形态变异中改变的肿瘤,星形胶质细胞瘤的形态与总体生存率的提高显着相关,强调提供综合的组织学和分子诊断的重要性。在分子定义的MN1改变的星形母细胞瘤中的组织学分级仍存在争议。在具有星形母细胞瘤形态的肿瘤中,小于15岁的患者中MN1改变状态的几率是15岁以上的人的10.5倍,女性是男性的9.4倍。根据观察数据,大体肿瘤切除似乎是主要的治疗方式,可以更好地控制疾病。

更新日期:2020-06-29
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