Beyond the Dura ― Massive Hyperostotic Response from a Convexity Meningioma: Systematic Review & Illustrative Case Example

Abstract Introduction Meningiomas account for approximately 41% of primary CNS neoplasms, a minority of which are associated with paracrine-induced cranial hyperostosis. We report a unique systematic review of this rare phenomenon alongside an illustrative case of a woman with 14 years of progressive pan-calvarial hyperostosis attributable to a bilateral parasagittal meningioma. Methods A novel case of massive cranial hyperostosis treated surgically and an associated PRISMA-compliant systematic review are presented. Searches of OVID MEDLINE and EMBASE (keywords “Hyperostosis” and “Meningioma”) on April 2, 2025 yielded 384 sources. All studies reporting calvarial hyperostosis associated with a convexity meningioma were included. Studies prior to the year 2000, non-human, or those that reported hyperostosis in other skull-base regions were excluded. Results A woman in her mid-40s presented with more than 14 years of enlarging pan-calvarial skull deformity predominantly involving the bilateral frontal bones. MRI revealed an enhancing midline mass obliterating the superior sagittal sinus consistent with meningioma. She underwent bifrontal craniotomy with en bloc hyperostotic bone removal and microsurgical meningioma resection. Reconstruction used dural grafting, titanium mesh cranioplasty, and scalp excision. Pathology confirmed a meningothelial meningioma with brain invasion (WHO Grade 2) with a solitary TRAF7 mutation. Systematic review yielded 25 studies (107 patients) showing a high prevalence of bifrontal bony involvement and meningothelial histology in cranial hyperostosis. Conclusion Mutations in TRAF7, a regulator of NF-κB, MAPK, and related pathways, are linked to calvarial hyperostosis and meningothelial histopathology. This systematic review and uniquely severe case highlight the potentially profound impact of TRAF7 mutation on paracrine hyperostosis.