Figure 1. Brain MRI showing tumor mass (TM) with cystic component (CC) and edema (T2-weighted MRI, left) in the lower brainstem and upper cervical cord. Mass with contrast enhancement (post-contrast T1-weighted MRI, middle and right).
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A teenage boy presented with 6 months of weight loss and difficulty swallowing. A contrast-enhancing mass in the brainstem and upper cervical cord was found which was suspicious of a symptomatic hemangioblastoma (Figure 1).
A pre-surgical cerebral angiogram was performed to investigate the vascular lesion and to attempt pre-operative embolization (Figure 2). A hypervascular posterior fossa tumor mass was confirmed with major vascular supply from the anterior spinal artery. The attempts for pre-surgical embolization were difficult (anterior spinal artery access) and had to be aborted.
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Figure 2. Posterior circulation cerebral angiogram. Right vertebral artery (R-VA) injection, mid- and late-arterial phase (A, B) and left VA injection mid- and late-arterial phase (C, D) showing filling of a vascular tumor mass (TM) fed by the anterior spinal artery (ASA). Basilar artery (BA). |
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Following, the intramedullary brainstem tumor was successfully resected via suboccipital craniotomy and upper cervical laminectomy. The patient developed some cystic pulmonary changes that were likely related to aspiration, but they have improved since. Otherwise, the post-surgical course was uneventful.
Two years following surgery, the patient had fully recovered, re-gained weight, and was a thriving adolescent boy (Figure 3).
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Figure 3. Two-year follow-up brain imaging (native head CT, left; non-contrast T1-weighted MRI, right) showing former suboccipital surgical access site, a slightly thinned upper cervical cord, and no residual tumor. |
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Treatment Considerations
Hemangioblastoma of the brain and spinal cord are rare, sporadic, and benign vascular tumors most commonly arising from the cerebellum1, 2, 3. An association with von-Hippel-Lindau disease exists which is inherited in an autosomal-dominant manner4.
Symptoms from the slowly growing tumor stem from mass effect caused by the cystic component and the tumor. Contrast-enhanced brain MRI shows a hypervascular tumor. However, the exact location of the tumor in terms of intra- or extramedullary or intra- and extramedullary can be difficult to determine on imaging and may only be clarified during surgery5.
Traditionally, surgical resection has been the treatment of choice2, 3. The utility of pre-surgical embolization and radio-surgery has been subject of clinical investigations, albeit a systematic approach has been difficult to realize due to the rare nature of the disease6, 7.
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REFERENCES:
- Nguyen HS, Doan N, Gelsomino M, et al. Intracranial hemangioblastoma – a SEER-based analysis 2004-2013. Oncotarget 2018; 9: 28009-015.
- Yin X, Li C, Li L, Duan H. Safety and efficacy of surgical treatment for brainstem hemangioblastoma: a meta-analysis. Neurosurg Rev. 2020 Apr 30. doi: 10.1007/s10143-020-01305-3. Epub ahead of print. PMID: 32356022.
- Giammattei L, Messerer M, Aghakhani N, et al. Surgical resection of medulla oblongata hemangioblastomas: outcome and complications. Acta Neurochir (Wien) 2016; 158: 1333-41.
- Lonser RR, Glenn GM, Walther M, et al. von Hippel-Lindau disease. Lancet. 2003;361:2059–67.
- Imagama S, Ito Z, Wakao N, et al. Differentiation of localization of spinal hemangioblastomas based on imaging and pathological findings. Eur Spine J 2001; 20: 1377-84.
- Bridges KJ, Jaboin JJ, Kubicky CD, Than KD. Stereotactic radiosurgery versus surgical resection for spinal hemangioblastoma: A systematic review. Clin Neurol Neurosurg. 2017; 154: 59-66.
- Sakamoto N, Ishikawa E, Nakai Y, et al. Preoperative endovascular embolization for hemangioblastoma in the posterior fossa. Neurol Med Chir (Tokyo) 2012; 52: 878-84.
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AUTHORS:
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NEUROLOGICAL SURGERY, P.C.
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