Anaplastic Intraventricular Meningioma with Rhabdoid Features: An Unusual Tumor with Usual Clinical Presentation
Related Experiment Video
Updated: Sep 2, 2025
Role of Diffusion MRI Tractography in Endoscopic Endonasal Skull Base Surgery
Published on: July 5, 2021
Preeti Agarwal1, Nancy Gupta1, Alok Srivastava2
1Department of Pathology, King George's Medical University, Lucknow, Uttar Pradesh, India.
Abstract:
Meningiomas are tumors arising from leptomeninges. Malignant counterpart of them is known as anaplastic meningioma which are WHO grade III tumors. Intraventricular location of these tumors is rare and is clinic-radiologically challenging. Histopathology and immunohistochemistry are confirmatory. We present case of a 27-year-old girl, who presented with usual symptoms of intraventricular mass in emergency. After shunt surgery, clinical diagnosis of ependymoma was formed with differential of high-grade glioma. Squash tissue was difficult to crush displaying tight clusters of spindle cells with necrosis in background. Definitive histology revealed high grade spindle cell neoplasm disposed in sheets with brisk and atypical mitosis. Only focal whorling pattern was seen. Large cells with eccentric cytoplasm, reminiscent of rhabdoid cells were also seen. Immunohistochemistry was positive for vimentin and EMA, negative for GFAP. Final diagnosis of Anaplastic meningioma was dispatched. The histological pattern of the present case, young age of presentation and presence of Rhabdoid cells make it unusual. Though rare but intraventricular meningiomas must also be kept in clinical radiological differentials apart from the usual ependymoma at this location.
Insights
Anaplastic meningioma, a rare WHO grade III tumor, can occur within the brain ventricles. This case highlights the diagnostic challenges and importance of considering intraventricular meningiomas in differential diagnoses.
Area of Science:
- Neuropathology
- Neuro-oncology
- Surgical Neurology
Background:
- Meningiomas originate from the meninges, with anaplastic meningioma representing the rare WHO grade III malignant form.
- Intraventricular meningiomas are exceptionally uncommon and pose significant diagnostic challenges due to their location and presentation.
- Accurate diagnosis relies on a combination of histopathology and immunohistochemistry.
Observation:
- A 27-year-old female presented with symptoms indicative of an intraventricular mass.
- Initial clinical and intraoperative diagnosis suggested ependymoma or high-grade glioma.
- Histopathological examination revealed a high-grade spindle cell neoplasm with necrosis, atypical mitosis, and rhabdoid-like cells.
Findings:
- Immunohistochemistry confirmed the diagnosis, showing positivity for vimentin and EMA, and negativity for GFAP.
- The final diagnosis was anaplastic meningioma, characterized by an unusual histological pattern, young patient age, and presence of rhabdoid cells.
- This case underscores the rarity of intraventricular anaplastic meningiomas.
Implications:
- Intraventricular meningiomas, though rare, should be included in the differential diagnosis for intraventricular masses, alongside common entities like ependymoma.
- Recognizing unusual histological features, such as rhabdoid morphology in this case, is crucial for accurate diagnosis.
- This case contributes to the understanding of rare intraventricular tumor presentations and diagnostic considerations.
