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Primary meningeal fibrosarcoma: a particular neuroradiological presentation
A Donnet1, D Figarella-Branger, F Grisoli
1Neurosurgical Department, CHU, Marseille, France. adonnet@ap-hm.fr
Abstract:
Primary meningeal fibrosarcoma is a rare malignant tumor of the central nervous system. About seventeen cases have been reported in the literature since 1980. We present the case of a patient whose initial neuroradiological manifestation of primary meningeal fibrosarcoma was an en plaque meningioma. Fifteen months later, he developed a dramatic clinical deterioration and coma. CT scan revealed a large frontal lesion. The mass was completely removed and histological examination was characteristic of meningeal fibrosarcoma.
Insights
Primary meningeal fibrosarcoma, a rare central nervous system tumor, can initially present as an en plaque meningioma. This case highlights its potential for delayed, aggressive progression and diagnosis.
Area of Science:
- Neuro-oncology
- Neurosurgery
- Pathology
Background:
- Primary meningeal fibrosarcoma is an exceptionally rare central nervous system malignancy.
- Fewer than twenty cases have been documented in medical literature since 1980.
- This tumor arises from the meninges, the membranes surrounding the brain and spinal cord.
Observation:
- The presented case initially manifested as an en plaque meningioma on neuroradiological imaging.
- The patient experienced a sudden decline in clinical status, progressing to coma fifteen months after initial presentation.
- A computed tomography (CT) scan identified a substantial frontal lobe lesion.
Findings:
- Surgical excision of the frontal mass was performed.
- Histopathological analysis confirmed the diagnosis of meningeal fibrosarcoma.
- This confirms the tumor's malignant nature and specific histological characteristics.
Implications:
- This case underscores the importance of considering rare differential diagnoses in neuro-oncology.
- The diagnostic challenge posed by meningioma-like initial presentations of meningeal fibrosarcoma is highlighted.
- Further research into the early detection and management of primary meningeal fibrosarcoma is warranted.
