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Table of Contents    
LETTER TO EDITOR
Year : 2014  |  Volume : 62  |  Issue : 2  |  Page : 228-229

Giant solid hemangioblastoma of the cerebellopontine angle: A technically challenging case


1 Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India
2 Department of Pathology, Section of Neuropathology, Christian Medical College, Vellore, Tamil Nadu, India
3 Department of Radiodiagnosis, Christian Medical College, Vellore, Tamil Nadu, India

Date of Submission19-Jan-2014
Date of Decision20-Jan-2014
Date of Acceptance06-Apr-2014
Date of Web Publication14-May-2014

Correspondence Address:
Vivek Joseph
Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.132450

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How to cite this article:
Nair BR, Joseph V, Chacko G, Keshava SN. Giant solid hemangioblastoma of the cerebellopontine angle: A technically challenging case. Neurol India 2014;62:228-9

How to cite this URL:
Nair BR, Joseph V, Chacko G, Keshava SN. Giant solid hemangioblastoma of the cerebellopontine angle: A technically challenging case. Neurol India [serial online] 2014 [cited 2021 May 15];62:228-9. Available from: https://www.neurologyindia.com/text.asp?2014/62/2/228/132450


Sir,

Excising a solid variety of hemangioblastoma in the cerebellopontine angle (CPA) is demanding due to the vascularity, narrow surgical corridor, and requirement for circumferential dissection with the proximity of the brainstem and cranial nerves. The authors describe a case of a large, vascular, solid hemangioblastoma of the CPA, excised successfully through a retro mastoid craniectomy, following embolization.

A 12 year old girl presented with gait ataxia, left-sided hearing impairment, and facial asymmetry of five months duration. She had House and Brackmann (H and B) grade three facial function and profound sensorineural hearing loss on the left side along with papilledema and cerebellar signs. Magnetic resonance imaging (MRI) brain showed a well enhancing 5 × 4.5 × 4.5 cm mass in the left CPA with internal cyst and peripheral flow voids and hydrocephalus [Figure 1]. Cerebral angiogram showed supply from left anterior inferior cerebellar artery, which was super selectively cannulated and the distal segments were embolized with 20% histacryl, resulting in 60% angiographic reduction in the tumor vascularity [Figure 2]. Embolization was immediately followed by a left retro-mastoid sub-occipital craniectomy and total excision with electrophysiological monitoring preserving the facial nerve. There was transient worsening of the facial paresis to H and B grade 4 and impairment of the gag reflex, which improved to preoperative state by tenth post-operative day. The histopathology was typical of a capillary hemangioblastoma [Figure 3]. One week post-operative computed tomography (CT) brain confirmed total excision of the tumor with reduction in hydrocephalus [Figure 4].
Figure 1: Preoperative MR axial T2W (a) and T1W (b-d) weighted Gadolinium images showing a 5 × 4.5 × 4.5 cm brilliantly enhancing mass with a superior cystic component and multiple flow voids within and at its periphery

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Figure 2: Right vertebral angiography, frontal view pre- and post-embolization images showing the vascular tumor fed by the left AICA and a 60% reduction in the tumor blush after embolization

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Figure 3: Histopathology image showing a partly circumscribed tumor composed of numerous capillary sized congested blood vessels and intervening stromal cells with mildly pleomorphic nuclei and moderate amounts of foamy to pale eosinophilic cytoplasm typical of a capillary hemangioblastoma

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Figure 4: Post-op axial contrast CT brain confirming total excision

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Hemangioblastomas account for 1.5%-2.5% of all intra-cranial tumors and 7%-12% of posterior fossa tumors. [1] Within the CPA, hemangioblastomas are extremely rare; only eight cases have been reported in the English literature to date. [2],[3],[4],[5],[6] Surgical excision is the definitive treatment for a hemangioblastoma. Long-term analysis after primary Stereotactic Radiosurgery (SRS) showed reduction in the benefit with time (70%, 61%, and 51% local control rates at 8, 10, and 15 years follow up), [7] and hence, SRS alone is reserved for patients who cannot safely undergo surgery for hemangioblastoma of less than 3 cm. Internal decompression is potentially dangerous for highly vascularized hemangioblastomas, where removal must be performed by en bloc resection after interruption of feeding arteries initially and draining veins later. Most of the reported cases have been operated as staged procedures or following radiotherapy or via a translabyrinthine approach. [2],[3],[4],[5],[6] Our method of preoperative embolization followed by immediate sub-occipital retro-mastoid approach and en bloc resection of the 5 × 4.5 cm CPA hemangioblastoma was safe with a good outcome.

 
  References Top

1.Hussein MR. Central nervous system capillary haemangioblastoma: The pathologist′s viewpoint. Int J Exp Pathol 2007;88:311-24.  Back to cited text no. 1
[PUBMED]    
2.Bush ML, Pritchett C, Packer M, Ray-Chaudhury A, Jacob A. Hemangioblastoma of the cerebellopontine angle. Arch Otolaryngol Head Neck Surg 2010;136:734-8.  Back to cited text no. 2
    
3.Dow GR, Sim DW, O′Sullivan MG. Excision of large solid haemangioblastomas of the cerebellopontine angle by a skull base approach. Br J Neurosurg 2002;16:168-71.  Back to cited text no. 3
    
4.Kamitani H, Hirano N, Takigawa H, Yokota M, Miyata H, Ohama E, et al. Attenuation of vascularity by preoperative radiosurgery facilitates total removal of a hypervascular hemangioblastoma at the cerebello-pontine angle: Case report. Surg Neurol 2004;62:238-244.  Back to cited text no. 4
    
5.Brackmann DE, Bartels LJ. Rare tumors of the cerebellopontine angle. Otolaryngol Head Neck Surg (1979) 1980;88:555-9.  Back to cited text no. 5
    
6.Rachinger J, Buslei R, Prell J, Strauss C. Solid haemangioblastomas of the CNS: A review of 17 consecutive cases. Neurosurg Rev 2009;32:37-48.  Back to cited text no. 6
    
7.Asthagiri AR, Mehta GU, Zach L, Li X, Butman JA, Camphausen KA, et al. Prospective evaluation of radiosurgery for hemangioblastomas in von Hippel-Lindau disease. Neuro Oncol 2010;12:80-6.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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