Supratentorial meningeal hemangioblastoma mimicking hypervascular meningioma
Presentation
Headache and left lower limb weakness.
Patient Data
Right high frontal extra-axial dural-based space-occupying lesion. It elicits a low signal on T1 WI and a remarkably high signal on T2 WI showing prominent flow voids and vivid post-contrast enhancement. The lesion shows a slight diffusion restriction. It's surrounded by large peritumoral vasogenic edema and exerts a positive mass effect in the form of compression of the right lateral ventricle.
Based on the remarkably high T2 signal of the lesion and the vivid post-contrast enhancement, a hypervascular subtype like angiomatous meningioma was radiologically presumed.
Pathology after excision
Gross picture: A well-defined mass with soft grayish tan and pink out section.
Microscopic picture: sections examined form S.O.L revealed tumor tissue, consistent of proliferating capillaries and irregular shaped widely dilated vascular spaces with perivascular hyalinosis. In between there were nests and lobules of stromal cells which were large, polygonal round and spindle in shape. Their cytoplasm were abundant and eosinophilic clear or vacuolated. The nuclei were small either central or eccentric. Mitotoic figures were inconspicuous. Necrosis was absent. The tumor had a delectable dural base. No evidence of frank anaplasia.
Diagnosis: Right fronto-pareital S.O.L. Excision biopsy specimen
Meningeal hemangioblastoma (WHO Grade 1)
Case Discussion
The patient had a negative family history of Von-Hipple disease. Genetic testing showed an absent mutation in the VHL tumor suppressor gene.
Hemangioblastomas are benign vascular tumors commonly found in the posterior fossa or the spinal cord, whereas supratentorial hemangioblastomas with meningeal involvement are extremely rare. Hemangioblastomas are common in Von-Hipple disease, but they might occur sporadically.
Supratentorial hemangioblastomas are usually solid and are mostly located in the frontal lobe 1.
Characteristic imaging findings include a bright signal on T2 WI, prominent flow voids, and vivid post-contrast enhancement. Also, peritumoral edema is usually observed.
Differential diagnosis includes angiomatous meningioma, hemangiopericytoma, and hypervascular metastatic lesions such as renal cell carcinoma metastasis.
Preoperative diagnosis can be helpful to decrease blood loss at surgery via preoperative embolization 2.
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