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Aggressive rhabdoid meningioma with osseous, papillary and chordoma-like appearance
Pinar Karabagli1, Hakan Karabagli, Guler Yavas
1Department of Pathology, Faculty of Medicine, Selcuk University, Konya, Turkey.
Abstract:
Meningiomas are the most common primary intracranial tumors. They are usually benign and slowly growing; however, they may show histologically malignant features categorizing them into grade II or III of World Health Organization (WHO) classification. Rhabdoid meningioma (RM) is an uncommon meningioma variant categorized as WHO grade III. The clinical course of RM is determined by local recurrences, invasion of adjacent brain and/or dura, widespread leptomeningeal dissemination, remote metastases and fatal clinical outcome. Herein we report a case with recurrent aggressive left occipital parasagittal region RM in which the patient initially declined radiation treatment. The tumor was resected four times in 5 years. Histopathological examination revealed a rhabdoid meningioma with metaplastic, papillary and chordoid differentiation. Six months after her fourth operation the patient died of progressive disease. RM is a rare subtype of malignant meningioma and the role of different adjuvant therapeutic options are still unknown. Clinical presentation, radiological features and pathologic findings of this uncommon tumor are discussed.
Insights
Rhabdoid meningioma (RM), a rare WHO grade III tumor, presents aggressive behavior with frequent recurrences and poor outcomes. This case highlights the challenges in managing this aggressive brain tumor despite multiple resections.
Area of Science:
- Neuro-oncology
- Pathology
- Neurosurgery
Background:
- Meningiomas are common primary intracranial tumors, typically benign but can be malignant (WHO grade II/III).
- Rhabdoid meningioma (RM) is a rare, aggressive WHO grade III variant with a poor prognosis.
- Clinical management of RM is challenging due to its aggressive nature and limited treatment options.
Observation:
- A case of recurrent aggressive left occipital parasagittal rhabdoid meningioma is presented.
- The patient underwent four tumor resections over five years, initially declining radiation therapy.
- Histopathology revealed rhabdoid features with metaplastic, papillary, and chordoid differentiation.
Findings:
- The patient experienced local recurrences, invasion, and ultimately died of progressive disease six months after the fourth surgery.
- Rhabdoid meningioma demonstrates aggressive behavior, including local recurrence, leptomeningeal dissemination, and metastasis.
- Despite aggressive surgical management, the prognosis for rhabdoid meningioma remains poor.
Implications:
- This case underscores the aggressive clinical course and poor prognosis associated with rhabdoid meningiomas.
- Further research is needed to determine the efficacy of adjuvant therapies for this rare malignant meningioma subtype.
- Understanding the clinical presentation, radiological features, and pathological findings is crucial for managing rhabdoid meningioma.
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