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<p>Meningiomas in pediatric and adolescent/young adult patients are poorly characterized biologically and clinically, and risk stratification is largely extrapolated from adult tumors. We analyze 293 tumors from patients aged 0–39 years using integrated histopathological and molecular profiling. Youth-onset meningiomas are enriched for <i>NF2</i> and <i>SMARCE1</i> alterations and exhibit a gain-dominated copy-number landscape, including recurrent chr17q gain, whereas canonical adult high-risk features, such as chr1p loss, lack prognostic significance. Adult-derived prognostic frameworks, including WHO grade, methylation-based stratification and integrated risk scores, fail to predict progression in patients ≤21 years of age. Tumors segregate into age-enriched epigenetic clusters defined by <i>SMARCE1</i>, <i>NF2</i> and <i>BAP1</i> alterations. Among <i>NF2</i>-altered tumors, patterns of Merlin inactivation, shaped by germline status and co-occurring copy-number variations, delineate biologically divergent subsets. In patients ≤21 years, extent of resection is the dominant predictor of outcome, while molecular features further refine risk assessment. These findings define pediatric and young adult meningiomas as a distinct molecular entity and support age-adapted risk refinement that integrates molecular features with strong clinical determinants.</p>

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Distinct molecular subgroups in pediatric and young-onset meningiomas require age-adapted risk stratification

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年7月14日

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  • Natalie Berghaus,
  • Arnault Tauziède-Espariat,
  • Thomas Hielscher,
  • Dilan Savran,
  • Daniel Schrimpf,
  • Kirsten Göbel,
  • Eric Stutheit-Zhao,
  • Lukas Friedrich,
  • Felix Keller,
  • Fuat Kaan Aras,
  • Filippo Nozzoli,
  • Dominik Sturm,
  • Christine L. White,
  • Simone Schmid,
  • Christian Mawrin,
  • Julia E. Neumann,
  • Till Acker,
  • Rudi Beschorner,
  • Christian Hartmann,
  • Irem Saribiyik,
  • Arda Inan,
  • Ayça Erşen-Danyeli,
  • Mariëtte E. G. Kranendonk,
  • Sybren L. N. Maas,
  • Eelke M. Bos,
  • Eleonora Aronica,
  • Saskia M. Peerdeman,
  • Nikki B. Thuijs,
  • Angelika Mühlebner,
  • Benno Kusters,
  • Wilfred F. A. den Dunnen,
  • Cinzia E. Lavarino,
  • Stéphanie Puget,
  • Jason Chiang,
  • Sonika Dahiya,
  • Melike Pekmezci,
  • Arie Perry,
  • Oluwadamilola Akanji,
  • Miriam Ratliff,
  • Christel Herold-Mende,
  • Sandro M. Krieg,
  • Wolfgang Wick,
  • Stefan M. Pfister,
  • Pieter Wesseling,
  • Andreas von Deimling,
  • Pascale Varlet,
  • Felix Sahm,
  • Philipp Sievers

摘要

Meningiomas in pediatric and adolescent/young adult patients are poorly characterized biologically and clinically, and risk stratification is largely extrapolated from adult tumors. We analyze 293 tumors from patients aged 0–39 years using integrated histopathological and molecular profiling. Youth-onset meningiomas are enriched for NF2 and SMARCE1 alterations and exhibit a gain-dominated copy-number landscape, including recurrent chr17q gain, whereas canonical adult high-risk features, such as chr1p loss, lack prognostic significance. Adult-derived prognostic frameworks, including WHO grade, methylation-based stratification and integrated risk scores, fail to predict progression in patients ≤21 years of age. Tumors segregate into age-enriched epigenetic clusters defined by SMARCE1, NF2 and BAP1 alterations. Among NF2-altered tumors, patterns of Merlin inactivation, shaped by germline status and co-occurring copy-number variations, delineate biologically divergent subsets. In patients ≤21 years, extent of resection is the dominant predictor of outcome, while molecular features further refine risk assessment. These findings define pediatric and young adult meningiomas as a distinct molecular entity and support age-adapted risk refinement that integrates molecular features with strong clinical determinants.