Neurointervention.  2023 Nov;18(3):209-213. 10.5469/neuroint.2023.00451.

Ruptured Medullary Hemangioblastoma Mimicking a Craniocervical Junction Dural Arteriovenous Fistula with a Pseudoaneurysm

• Park S
• Kwon B
• Lee DH
• Ahn JS
• Song Y

• KMID: 2547285
• DOI: http://doi.org/10.5469/neuroint.2023.00451

Abstract

Hemangioblastomas (HBMs) are rare vascular tumors commonly located in the posterior fossa of adults. A mid-50s patient presented with sudden unconsciousness. Computed tomography scans revealed acute hemorrhages around the posterior fossa, predominantly in the subarachnoid space. Digital subtraction angiography (DSA) revealed an 8-mm round lesion filled with contrast agent, fed by the C1 segmental artery of the left vertebral artery (VA), showing early venous drainage to the spinal cord and brainstem. Emergent embolization was attempted under suspicion of a ruptured dural arteriovenous fistula, resulting in parent artery occlusion due to feeder selection failure. Follow-up DSA after a month depicted a persistent aneurysm via collaterals from both VAs. Consequently, the decision was made to proceed with surgical intervention, leading to the resection of the lesion, confirming its diagnosis as a HBM through histological examination. This case underscores the potential for misdiagnosis when HBMs with an intratumoral shunt mimic vascular shunt lesions.

Keyword

Aneurysm; Dural arteriovenous fistula; Hemangioblastoma

Figure

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  Fig. 1. (A) Noncontrast head computed tomography (CT) identified acute subarachnoid (black arrows), and subdural hemorrhages (black arrowheads) around the posterior fossa. (B) CT angiography highlighted a small, contrast-enhancing lesion situated on the dorsolateral aspect of the medulla (white arrow). (C) Left vertebral arteriography depicted a circular contrast-enhanced lesion (arrow) neighboring the left vertebral artery (VA) at the craniocervical junction, with an early draining vein extending downward towards the spinal cord (arrowheads). (D) Even after segmental occlusion of the left VA, the contralateral vertebral arteriography still revealed the same lesion (arrow) through delicate pial collaterals encircling the medulla. (E) After segmental occlusion of the left VA, the aneurysm-resembling lesion also manifested enhancement on the contrast-enhanced T1-weighted image (arrow). (F, G) One-month post-intervention, a follow-up digital subtraction angiography depicted a persistent presence of the aneurysm (arrow) in both VA angiograms. (H) Gross images captured intraoperatively. The mass (arrowheads) was received its blood supply from delicate pial arteries, draining into an arterialized vein (arrow). (I) The nuclei of the mesenchymal cells varied in size (H&E staining, ×400). (J) Inhibin staining was positive, showing the strongly cytoplasmic reactivity (Inhibin-α staining, ×400).

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