Presentation
Occipital headache that wakes the patient up at night and worsens with Valsalva. Vomiting and dizziness.
Patient Data
Non-contrast brain CT:
Hypodense intra-axial infratentorial mass with cystic appearance and soft tissue density nodule in the right cerebellar hemisphere extending to the left hemisphere. Perilesional edema. Mass effect, deforming the 4th ventricle. No hydrocephalus. The ventricular system and cisternal spaces are normal.
Contrast brain CT:
No significant enhancement is observed after contrast administration except for a hyper-enhancing solid anteromedial and apical nodule.
T1: hypointense lesion with an isointense solid pole.
T1 C+: non-enhancing cystic wall with a hyperintense nodule.
T2: hyperintense mass with an isointense solid pole. Perilesional edema.
DWI/ADC: facilitated diffusion, suggesting a cystic component.
FLAIR: hyperintense nodule. The mass content is not suppressed in the FLAIR sequence, and therefore, it rules out cerebrospinal fluid. Perilesional edema.
Volumetric MRI sequence (perfusion CT): hyperintense nodule with elevated perfusion.
Case Discussion
Hemangioblastomas are rare WHO grade I central nervous system tumors. They are most frequently found in the posterior fossa, followed by the brain stem and spinal cord, and are rare in the supratentorial region. They are most common among white men between the ages of 65-69, and about 30% of these tumors are related to von Hippel-Lindau syndrome. Most tumors are cystic lesions with mural nodules, but can also appear as solid or cystic tumors. Due to their high vascularity, it's important to differentiate hemangioblastomas from other vascular tumors and clear cell meningiomas.
Despite being benign, they can lead to substantial neurological complications and even mortality due to their localization, mass effect and potential for multiplicity. Surgery stands as an effective treatment with positive outcomes, and radiosurgery may be necessary for patients with partially removed or nonresectable tumors, or if the patient is unable to undergo surgery due to other health conditions.
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