Lateral Ventricular Lesion as a Presentation of Von Hippel-Lindau Syndrome: A Case Study

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Von Hippel-Lindau disease is an autosomal dominant disorder caused by mutations in the VHL tumor suppressor gene on chromosome 3.
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The disease is characterized by the formation of vascular-rich tumors or cysts in multiple organ systems, including the central nervous system.
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A 54-year-old man diagnosed with VHL syndrome presented with an intracranial lateral ventricular hemangioblastoma and metastatic renal cell carcinoma.
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Postoperative complications included extensive intracranial hemorrhage, leading to fatal brainstem hemorrhage due to the hemangioblastoma's high vascularity.
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The case highlights the diagnostic challenges of VHL syndrome and the surgical risks associated with central nervous system lesions.

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