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Pediatric rhabdoid meningioma: a morphological, immunohistochemical, ultrastructural and molecular case study
Anna Maria Buccoliero1, Francesca Castiglione, Duccio Rossi Degl'Innocenti
1Department of Biomedicine, Careggi Hospital, Florence, Italy. ambuccoliero@unifi.it
Abstract:
Rhabdoid meningioma is an uncommon meningioma variant categorized as WHO grade III. The majority of cases occur in adulthood. Herein, we describe a right fronto-temporal rhabdoid meningioma affecting a 3-year-old boy. The lesion measured approximately 4 cm in diameter and incorporated the ipsilateral middle cerebral artery. Sub-total surgical excision of the mass was performed. Histologically, the tumor was mainly composed of globoid plump cells with inclusion-like eosinophilic cytoplasm, peripheral nuclei, prominent nucleoli and occasional intra-nuclear cytoplasmic pseudo-inclusion. The cells appeared in many areas loosely arranged and focally disclosed a papillary architecture. At immunohistochemistry, the tumor cells were EMA, vimentin, HHF35, PgR, INI-1 and p53 positive. The proliferative index (Mib-1) was 15% in the most positive areas. Ultrastructurally, tumoral cells showed an abundant cytoplasm, which was filled with numerous intermediate filaments. Desmosomal junctions were seen. RT-PCR revealed the presence of NF2 gene expression. Molecular study did not indicate alterations of the INI-1 gene, whereas it showed the presence of Pro72Arg in exon 4 at heterozygous state in the TP53 gene. Morphologic features along with immunohistochemical, ultrastructural and molecular results were consistent with the diagnosis of rhabdoid meningioma. The patient was treated with chemotherapy. The lesion remained stable after 33 months of follow-up. Rhabdoid meningiomas rarely occur in children. Owing to its rarity, each new case should be recorded to produce a better clinical, pathological, molecular, prognostic and therapeutic characterization of this lesion.
Insights
This case report details a rare rhabdoid meningioma in a 3-year-old boy, highlighting its unique presentation and molecular characteristics. Further case documentation is crucial for understanding this aggressive brain tumor in children.
Area of Science:
- Neuro-oncology
- Pediatric pathology
- Molecular diagnostics
Background:
- Rhabdoid meningioma is a rare, aggressive WHO grade III tumor, typically seen in adults.
- Pediatric rhabdoid meningiomas are exceptionally uncommon, necessitating detailed case reporting.
Observation:
- A 3-year-old boy presented with a large right fronto-temporal rhabdoid meningioma involving the middle cerebral artery.
- Histopathology revealed characteristic globoid cells with eosinophilic cytoplasm and papillary architecture.
- Immunohistochemistry showed positivity for EMA, vimentin, HHF35, PgR, INI-1, and p53, with a 15% Mib-1 proliferation index.
Findings:
- Ultrastructural analysis confirmed abundant intermediate filaments and desmosomal junctions.
- Molecular studies revealed NF2 gene expression and a heterozygous Pro72Arg variant in the TP53 gene, with no INI-1 alterations.
- The tumor was sub-totally excised and treated with chemotherapy, showing stable disease at 33 months.
Implications:
- This case contributes to the limited understanding of pediatric rhabdoid meningiomas.
- Detailed characterization of rare pediatric tumors is vital for improving clinical management and therapeutic strategies.
- Further research into the molecular underpinnings of pediatric rhabdoid meningiomas may reveal novel therapeutic targets.
