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Atypical Intraparenchymal Meningioma with YAP1-MAML2 Fusion in a Young Adult Male: A Case Report and Mini Literature
Alisa Nobee1, Mei Xu2, Anjali Seth1
1Department of Pathology and Laboratory Medicine, Temple University Hospital, Philadelphia, PA 19140, USA.
Abstract:
Oncogenic Yes-associated protein (YAP) 1 fusions have been recently identified in several cases of meningioma mostly involving pediatric patients. The meningiomas harboring YAP1-MAML2, which is the most frequent fusion subtype, exhibit activated YAP1 signaling and share similarities with NF2 (neurofibromatosis type 2 gene) mutant meningiomas. We reported a rare case of atypical intraparenchymal meningioma with YAP1-MAML2 fusion in a 20-year-old male. The patient presented with an episode of seizure without a medical history. MRI revealed a lesion in the right temporal lobe without extra-axial involvement. The radiological and morphological findings, however, were indistinctive from other intracranial diseases, e.g., vascular malformation and glioma. Immunohistochemical results confirmed the presence of abundant meningothelial cells in the tumor and indicated brain invasion, supporting the diagnosis of atypical intraparenchymal meningioma. Targeted RNA fusion analysis further identified a YAP1-MAML2 rearrangement in the tumor. Non-dural-based intraparenchymal meningiomas are uncommon, and the careful selection of specific tumor markers is crucial for an accurate diagnosis. Additionally, the detection of the fusion gene provides valuable insights into the oncogenic mechanism of meningioma.
Insights
This study reports a rare intraparenchymal meningioma with a YAP1-MAML2 fusion in a young adult. This finding offers insights into meningioma oncogenesis and diagnosis.
Area of Science:
- Neuro-oncology
- Molecular Pathology
Background:
- Oncogenic Yes-associated protein (YAP) 1 fusions, particularly YAP1-MAML2, are increasingly recognized in meningiomas, especially in pediatric cases.
- These fusions activate YAP1 signaling and share characteristics with NF2-mutant meningiomas.
Approach:
- A rare case of atypical intraparenchymal meningioma with YAP1-MAML2 fusion in a 20-year-old male is presented.
- Diagnosis involved MRI, immunohistochemistry for meningothelial cells and brain invasion, and targeted RNA fusion analysis.
Key Points:
- The tumor was intraparenchymal, lacking extra-axial involvement, with non-distinctive radiological and morphological findings.
- Immunohistochemistry confirmed meningothelial differentiation and brain invasion.
- Targeted RNA sequencing identified the YAP1-MAML2 fusion.
Conclusions:
- Non-dural-based intraparenchymal meningiomas are uncommon and require specific diagnostic markers.
- Detection of the YAP1-MAML2 fusion provides critical information on meningioma oncogenic mechanisms.
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