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Falcine meningioma in Von Hippel-Lindau disease: An unusual association
Domenico Murrone1, Bruno Romanelli1, Nicola Montemurro1
1Unit of Neurosurgery, "Di Venere" City Hospital, Bari, Italy.
Background:
Von Hippel-Lindau (VHL) disease is an autosomal dominant condition characterized by formation of multiple benign and malignant tumors. In this disease supratentorial lesions are rare and no falcine meningioma has been previously reported. Differential diagnosis is very difficult and the histopathological examination is the definitive method for diagnosis.
Case Description:
A patient with VHL underwent a suboccipital craniotomy for removal of cerebellar hemangioblastoma and after 2 years magnetic resonance imaging (MRI) showed an iperintense solid mass located at posterior part of the falx. Histological diagnosis revealed meningioma.
Conclusion:
The only case in the literature of falcine meningioma in a patient with Von Hippel-Lindau disease, discovered during radiological follow-up, is described and a surgical management is proposed.
Insights
Von Hippel-Lindau (VHL) disease rarely presents with supratentorial lesions. This study details the first reported case of a falcine meningioma in a VHL patient, discovered via MRI.
Area of Science:
- Neuro-oncology
- Genetics
- Neurosurgery
Background:
- Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder associated with diverse tumor development.
- Supratentorial lesions are uncommon in VHL disease, with falcine meningiomas previously unreported.
- Accurate diagnosis often necessitates histopathological examination due to challenging differential diagnoses.
Observation:
- A patient with VHL disease developed a falcine meningioma 2 years post-surgery for a cerebellar hemangioblastoma.
- Magnetic resonance imaging (MRI) revealed an iperintense solid mass at the posterior falx.
- Histological analysis confirmed the mass as a meningioma.
Findings:
- This report presents the unique case of a falcine meningioma in a patient diagnosed with Von Hippel-Lindau disease.
- The meningioma was incidentally discovered during routine radiological follow-up.
- This represents the first documented instance of a falcine meningioma associated with VHL disease in medical literature.
Implications:
- Highlights the importance of vigilant radiological surveillance in VHL patients, even for rare tumor locations.
- Suggests that meningiomas, though rare, should be considered in the differential diagnosis of supratentorial lesions in VHL patients.
- Proposes surgical management strategies for this rare presentation, contributing to VHL disease management guidelines.
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