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Journal of Craniovertebral Junction and Spine
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   Table of Contents  
REVIEW ARTICLE
Year : 2016  |  Volume : 7  |  Issue : 4  |  Page : 197-200  

Purely extradural spinal nerve root hemangioblastomas


1 Department of Neurosurgery, Vocational School of Health Services, Acıbadem University, Istanbul, Turkey
2 Department of Neurosurgery, Acıbadem University School of Medicine, Istanbul, Turkey
3 Department of Orthopedic Surgery-Spine Center, University of California at San Francisco, CA, USA
4 Department of Neurosurgery, Acıbadem Maslak Hospital, Istanbul, Turkey
5 Department of Pathology, Acıbadem University School of Medicine, Istanbul, Turkey
6 Department of Orthopedics and Traumatology, Acıbadem University School of Medicine, Istanbul, Turkey

Date of Web Publication2-Nov-2016

Correspondence Address:
Murat Hamit Aytar
Special Family Hospital, Department of Neurosurgery, Talatpaşa Blv. Begonya Street, No:7-9, 34590, Istanbul
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-8237.193255

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   Abstract 

Spinal nerve root hemangioblastomas present mostly as intradural-extradurally. Purely extradural spinal nerve root hemangioblastoma is a very rare entity. In this study, we aimed to analyze epidemiological perspectives of purely extradural spinal nerve root hemangioblastomas presented in English medical literature in addition to our own exemplary case. PubMed/MEDLINE was searched using the terms “hemangioblastoma,” “extradural,” “spinal,” and “nerve root.” Demographical variables of age, gender, concomitant presence of von Hippel–Lindau (VHL) disease; spinal imaging and/or intraoperative findings for tumor location were surveyed from retrieved articles. There are 38 patients with purely extradural spinal nerve root hemangioblastoma. The median age is 45 years (range = 24–72 years). Female:male ratio is 0.6. Spinal levels for purely extradural spinal nerve root hemangioblastomas, in order of decreasing frequency, are thoracic (48.6%), cervical (13.5%), lumbar (13.5%), lumbosacral (10.8%), sacral (8.1%), and thoracolumbar (5.4%). Concomitant presence of VHL disease is 45%. Purely extradural spinal nerve root hemangioblastomas are very rare and can be confused with other more common extradural spinal cord tumors. Concomitant presence of VHL disease is observed in less than half of the patients with purely extradural spinal nerve root hemangioblastomas. Surgery is the first-line treatment in these tumors.

Keywords: Extradural; hemangioblastoma; spine; surgery; von Hippel–Lindau.


How to cite this article:
Aytar MH, Yener U, Ekşi MS, Kaya B, Özgen S, Sav A, Alanay A. Purely extradural spinal nerve root hemangioblastomas. J Craniovert Jun Spine 2016;7:197-200

How to cite this URL:
Aytar MH, Yener U, Ekşi MS, Kaya B, Özgen S, Sav A, Alanay A. Purely extradural spinal nerve root hemangioblastomas. J Craniovert Jun Spine [serial online] 2016 [cited 2018 Nov 19];7:197-200. Available from: http://www.jcvjs.com/text.asp?2016/7/4/197/193255


   Introduction Top


Hemangioblastoma is a benign hypervascular tumor seen concomitant with von Hippel–Lindau (VHL) disease in ¼ of the patients and mostly presents in the posterior cranial fossa and the spinal cord (¾ intramedullary, ¼ extramedullary).[1],[2] Spinal nerve root hemangioblastomas present mostly as intradural-extradurally. Purely extradural spinal nerve root hemangioblastoma is a very rare entity. In this study, we aimed to analyze epidemiological perspectives of purely extradural spinal nerve root hemangioblastomas presented in English medical literature in addition to our own exemplary case.


   Materials and Methods Top


PubMed/MEDLINE was searched using the terms “hemangioblastoma,” “extradural,” “spinal,” and “nerve root.” Patient demographical variables of age, gender, concomitant presence of VHL disease; spinal imaging and/or intraoperative findings for tumor location were surveyed. Spinal nerve root hemangioblastoma cases with intradural origins were excluded from the final analysis.


   Results Top


Thirty-eight patients with purely extradural spinal nerve root hemangioblastoma were retrieved from literature survey and our own case.[1],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21], The median age is 45 years (range = 24–72 years). The female:male ratio is 0.6. Most common spinal location for purely extradural hemangioblastoma is thoracic level (48.6%). Other spinal locations are cervical (13.5%), lumbar (13.5%), lumbosacral (10.8%), sacral (8.1%), and thoracolumbar (5.4%). Concomitant presence of VHL disease is seen in 45% of the patients [Table 1].
Table 1: Purely extradural spinal nerve root hemangioblastoma cases presented in the literature

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Exemplary case

A 43-year-old man was admitted with dorsal and low back pain with progressive bilateral leg weakness. On neurological examination, he had 1/5 paraparesia and right foot clonus (2–3 beats). Whole spinal magnetic resonance imaging (MRI) was obtained, which depicted a mass lesion at thoracic 7–8 levels. The mass lesion enhanced intensely following intravenous (IV) contrast administration and it was located in the right posterolateral side of the spinal canal with some invasion into the same side intervertebral foramen [Figure 1]. The patient underwent surgery. Total laminectomy of T7–T8 and en bloc removal of mass lesion was performed by posterior approach. The mass lesion was adherent to the right T7 nerve root with slight infiltration to the dura mater [Figure 2]. No intraoperative complication occurred. Postoperative neurological status of the patient was same as the preoperative condition. The histopathology of the specimen was compatible with grade-I hemangioblastoma (WHO 2007 classification) [Figure 3]. Neither he nor his family had a history of VHL disease. At the fourth postoperative day, he was discharged with ongoing physical rehabilitation.
Figure 1: Homogenous enhancement is observed at right T7 nerve root location displacing the spinal cord to the left side of the spinal canal

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Figure 2: A vascular lesion is observed just over the dura. The lesion is adherent to the dura, yet not invading it

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Figure 3: Encapsulated hemangioblastoma nodule attached to fibrous tissue (a - H and E, ×0.8). Tumor tissue showing thin-walled blood vessels of various diameters (b - H and E, ×4.2). High magnification reveals cells with cytoplasmic vacuolation typical for a hemangioblastoma (c - H and E, ×44.3)

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   Discussion Top


Lindau defined the hemangioblastoma in 1926.[12],[22] Origin of hemangioblastomas could not have been defined, yet. Hemangioblastomas present not only in the nervous system but also in other organs systems, so glial cells might not be the source of the tumor cells. Both mesodermal and neuroectodermal tissues have been proposed to be possible sources of hemangioblastoma, recently.[12]

Hemangioblastomas are sometimes seen concomitant with VHL disease, which is an autosomal dominant disorder of tumor suppressor gene, VHL.[13] More than half of the patients with spinal nerve root hemangioblastoma were found to have concomitant VHL disease.[11] However, in this study, concomitant VHL disease rate has been found as only 45%.

Spinal hemangioblastomas compose 1.6–5.8% of all spinal cord tumors and mostly present intramedullary in the dorsal aspect of cervical and thoracic spinal cord segments.[1],[11] Sometimes, spinal hemangioblastomas present in both intramedullary and extramedullary (6–8% of spinal hemangioblastomas). Remaining rare locations are cauda equina, filum terminale, and proximal spinal nerve roots.[1],[11] The most common site for purely extradural spinal nerve root hemangioblastomas is thoracic level (48.6%).

On MRI, spinal hemangioblastomas appear as hypo- to isointense on both T1- and T2-weighted scans with near homogeneous enhancement following IV contrast material administration. In the presence of intratumoral hemorrhage, intensities may differ on T1- and T2-weighted MRI.[11] Presence of prominent vessels in the subarachnoid space is diagnostic for hemangioblastomas.[8],[23] Opposite to cystic components found in intracranial and intramedullary hemangioblastomas; extramedullary hemangioblastomas are solid in nature.[24] Angiography could be useful to depict vascularity of the tumor, and even preoperative embolization can be conveyed where needed.[11]

In differential diagnosis; meningioma, schwannoma, ependymoma, paraganglioglioma, and metastasis (especially renal cell carcinoma, due to its high vascularity) should be considered.[12]

Management of spinal nerve root hemangioblastomas starts with appropriate preoperative imaging to prevent massive intra-operative bleeding. It should be kept in mind that hemangioblastomas may show invasion into surrounding soft and bony tissue. Surgical experience is the mainstay of success in excision of these tumors.[13] There is a tendency to recur especially in cases with an inadequate amount of resection.[12]


   Conclusion Top


Purely extradural spinal nerve root hemangioblastomas are very rare, and they might be confused with other more common extradural spinal tumors. Concomitant presence of VHL disease is observed in less than half of the patients with purely extradural spinal nerve root hemangioblastoma. Surgery, with careful preoperative planning, is the first line treatment in these tumors.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest

 
   References Top

1.
Law EK, Lee RK, Griffith JF, Siu DY, Ng HK. Spinal nerve root haemangioblastoma associated with reactive polycythemia. Case Rep Radiol 2014;2014:798620.  Back to cited text no. 1
    
2.
Senjaya F, Midha R. Thoracic nerve root hemangioblastoma – A diagnostic rarity. World Neurosurg 2012;78:88-9.  Back to cited text no. 2
    
3.
Smith RA, Estridge MN. Extradural spinal hemangioblastomas. Review of the literature and report of a case. Bull Los Angel Neuro Soc 1963;28:79-84.  Back to cited text no. 3
    
4.
Murota T, Symon L. Surgical management of hemangioblastoma of the spinal cord: A report of 18 cases. Neurosurgery 1989;25:699-707.  Back to cited text no. 4
    
5.
Chazono M, Shiba R, Funasaki H, Soshi S, Hattori A, Fujii K. Hemangioblastoma of the L-5 nerve root. Case illustration. J Neurosurg 1999;90 1 Suppl: 160.  Back to cited text no. 5
    
6.
Chu BC, Terae S, Hida K, Furukawa M, Abe S, Miyasaka K. MR findings in spinal hemangioblastoma: Correlation with symptoms and with angiographic and surgical findings. AJNR Am J Neuroradiol 2001;22:206-17.  Back to cited text no. 6
    
7.
Hermier M, Cotton F, Saint-Pierre G, Jouvet A, Ongolo-Zogo P, Fischer G, et al. Myelopathy and sciatica induced by an extradural S1 root haemangioblastoma. Neuroradiology 2002;44:494-8.  Back to cited text no. 7
    
8.
Escott EJ, Kleinschmidt-DeMasters BK, Brega K, Lillehei KO. Proximal nerve root spinal hemangioblastomas: Presentation of three cases, MR appearance, and literature review. Surg Neurol 2004;61:262-73.  Back to cited text no. 8
    
9.
Gläsker S, Berlis A, Pagenstecher A, Vougioukas VI, Van Velthoven V. Characterization of hemangioblastomas of spinal nerves. Neurosurgery 2005;56:503-9.  Back to cited text no. 9
    
10.
Kern M, Naeini R, Lehmann TN, Benndorf G. Imaging of a thoracic spinal nerve haemangioblastoma by three-dimensional digital angiography. J Clin Neurosci 2006;13:929-32.  Back to cited text no. 10
    
11.
Purandare HR, Misra BK. Thoracic nerve root hemangioblastoma: A rare cause of posterior mediastinal mass. World Neurosurg 2012;78:192.E1-3.  Back to cited text no. 11
    
12.
Mitchell A, Scheithauer BW, Wharen RE, Franck J, Chan K. Hemangioblastoma of spinal nerve: A report of six cases. Clin Neuropathol 2013;32:91-9.  Back to cited text no. 12
    
13.
Sun HI, Özduman K, Usseli MI, Özgen S, Pamir MN. Sporadic spinal hemangioblastomas can be effectively treated by microsurgery alone. World Neurosurg 2014;82:836-47.  Back to cited text no. 13
    
14.
Román-de Aragón M, Márquez T, Isla A, Gómez-de la Riva A. Hemangioblastoma of the right L4 nerve root with radicular extraforaminal involvement. Neurocirugia (Astur) 2014;25:286-9.  Back to cited text no. 14
    
15.
Zakaria R, Crooks D, Osman-Farah J. Reply to: Clinical Neuropathology 2013;32:91-9. Spinal nerve root hemangioblastoma may be predominantly extradural. Clin Neuropathol 2014;33:157-9.  Back to cited text no. 15
    
16.
Laviv Y, Rappaport ZH. Cord compression due to extradural thoracic nerve root hemangioblastoma. Br J Neurosurg 2015;29:281-4.  Back to cited text no. 16
    
17.
Doyle LA, Fletcher CD. Peripheral hemangioblastoma: Clinicopathologic characterization in a series of 22 cases. Am J Surg Pathol 2014;38:119-27.  Back to cited text no. 17
    
18.
Tarlov IM. Spinal extradural hemangioblastoma roentgenographically visualized with diodrast at operation and successfully removed. Radiology 1947;49:717-23.  Back to cited text no. 18
    
19.
Higgins JN, Lammie GA, Savy LE, Taylor WJ, Stevens JM. Intraosseous vertebral haemangioblastoma: MRI. Neuroradiology 1996;38 Suppl 1:S107-10.  Back to cited text no. 19
    
20.
Lee DK, Choe WJ, Chung CK, Kim HJ. Spinal cord hemangioblastoma: Surgical strategy and clinical outcome. J Neurooncol 2003;61:27-34.  Back to cited text no. 20
    
21.
Choudhury T, Jahan S, Kamal M, Hossain MM, Khan ZR. Von Hippel-Lindau disease in a pregnant lady. Mymensingh Med J 2012;21:184-7.  Back to cited text no. 21
    
22.
Lindau A. Studies on cerebellar cysts: Structure, pathogenesis and relationship with retinal angiomatosis. Acta Pathol Microbiol Scand 1926;1:1-128.  Back to cited text no. 22
    
23.
Sawyer B, Heng R, Ferris N. Typical magnetic resonance features of a haemangioblastoma, an uncommon nerve root lesion: Case report and literature review. Australas Radiol 2007;51:B28-30.  Back to cited text no. 23
    
24.
Browne TR, Adams RD, Roberson GH. Hemangioblastoma of the spinal cord. Review and report of five cases. Arch Neurol 1976;33:435-41.  Back to cited text no. 24
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

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