Central nervous system neuroepithelial tumors with MN1 -alteration: an individual patient data meta-analysis of 73 cases
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ORIGINAL ARTICLE
Central nervous system neuroepithelial tumors with MN1‑alteration: an individual patient data meta‑analysis of 73 cases Wanwan Chen1,2 · Yu Yang Soon3 · Patricia Diana Pratiseyo4 · Ratna Sutanto5 · Lutfi Hendriansyah6 · Chik Hong Kuick2 · Kenneth T. E. Chang2,7 · Char Loo Tan2,8,9 Received: 16 May 2020 / Accepted: 23 June 2020 © The Japan Society of Brain Tumor Pathology 2020
Abstract 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. Keywords MN1 · Astroblastoma · Primitive neuroepithelial tumor · CNS HGNET-MN1 · Meta-analysis
Introduction Central nervous system high grade neuroepithelial tumor with MN1 alteration (CNS HGNET-MN1) is a recently described tumor type characterized by recurrent genetic alteration involving MN1. This tumor type was first Electronic supplementary material The online version of this article (https://doi.org/10.1007/s10014-020-00372-0) contains supplementary material, which is available to authorized users. * Char Loo Tan [email protected] 1
Department of Pathology, The Second Affiliated Hospital and Yuying Children’s Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China
2
Department of Pathology and Laboratory Medicine, KK Women’s and Children’s Hospital, Singapore, Singapore
3
Department of Radiation Oncology, National University Health System, Singapore, Singapore
4
Department of Pathology, Siloam Hospital Lippo Village Karawaci, Tangerang, Indonesia
described in 2016 by Sturm et al. from their study of 323 CNS primitive neuroectodermal tumors (PNET) using DNA methylation profiling [16]. The authors also found that 16 out of 23 tumors histologically diagnosed as astroblastoma clustered with CNS HGNET-MN1, suggesting that at least a subset of as
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