To investigate the clinicopathological features, immunophenotype, diagnosis, and differential diagnosis of meningioma with rosettes.

The clinical, pathological, and imaging data of an atypical meningioma with rosettes with skull invasion were analyzed and the relevant literature was reviewed.

A 56-year-old male patient underwent a head MRI scan showing an irregular massive soft tissue shadow under the top skull. On pathological examination, the mass measured 11.0 cm×9.5 cm×1.5 cm, which destroyed the bone tissue. Microscopically, the tumor was composed of spindle cells and epithelioid cells. It was characterized by the diffuse distribution of rosettes, with the eosinophilic and oligocellular zone in the center. Some of rosettes were composed of elongated slender tumor cells, and there was no obvious vessel-like cavum structure in it. Immunohistochemical staining revealed that the tumor cells were positive for EMA, SSTR2a, Vimentin, PR, E-cadherin, D2-40, and P53, but negative for GFAP, S-100, STAT6, CD99, Bcl-2, Fli-1, NSE, Syn, CD45, Melanoma, and SATB2. Second-generation sequencing revealed a mutation in the fourth exon of the NF2 gene and MGMT promoter methylation.

Meningioma with rosettes is rare, and its diagnosis mainly depends on the presence of typical meningoepithelial differentiation region and immunohistochemistry. When rosette-like structure appears in poorly differentiated cases, meningioma should be considered.