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Central nervous system neuroepithelial tumors with MN1-alteration: an individual patient data meta-analysis of 73 cases

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 lesion...

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Bibliographic Details
Published in:Brain tumor pathology 2020-10, Vol.37 (4), p.145-153
Main Authors: Chen, Wanwan, Soon, Yu Yang, Pratiseyo, Patricia Diana, Sutanto, Ratna, Hendriansyah, Lutfi, Kuick, Chik Hong, Chang, Kenneth T. E., Tan, Char Loo
Format: Article
Language:English
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Summary: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.
ISSN:1433-7398
1861-387X
DOI:10.1007/s10014-020-00372-0