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

Brain Tumor Pathol. 2020 Oct;37(4):145-153. doi: 10.1007/s10014-020-00372-0. Epub 2020 Jun 29.

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: Astroblastoma; CNS HGNET-MN1; MN1; Meta-analysis; Primitive neuroepithelial tumor.

Publication types

  • Case Reports
  • Meta-Analysis
  • Systematic Review

MeSH terms

  • Age Factors
  • Central Nervous System Neoplasms* / diagnostic imaging
  • Central Nervous System Neoplasms* / mortality
  • Central Nervous System Neoplasms* / pathology
  • Central Nervous System Neoplasms* / surgery
  • Child
  • Female
  • Humans
  • Neoplasms, Neuroepithelial* / diagnostic imaging
  • Neoplasms, Neuroepithelial* / mortality
  • Neoplasms, Neuroepithelial* / pathology
  • Neoplasms, Neuroepithelial* / surgery
  • Neuroectodermal Tumors, Primitive* / diagnosis
  • Neuroectodermal Tumors, Primitive* / pathology
  • Neuroectodermal Tumors, Primitive* / surgery
  • Prognosis
  • Sex Factors
  • Trans-Activators
  • Tumor Suppressor Proteins

Substances

  • MN1 protein, human
  • Trans-Activators
  • Tumor Suppressor Proteins