Article

Spinal Cord Hemangioblastomas in von Hippel-Lindau Disease: Management of Asymptomatic and Symptomatic Tumors

Department of Neurosurgery, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul 120-752, Korea. .
Yonsei medical journal (Impact Factor: 1.29). 11/2012; 53(6):1073-80. DOI: 10.3349/ymj.2012.53.6.1073
Source: PubMed

ABSTRACT

Purpose
Standard treatment of asymptomatic spinal cord hemangioblastoma in von Hippel-Lindau (VHL) disease has yet to be established. The purpose of this study was to propose guidelines for the treatment of asymptomatic spinal cord hemangioblastomas in VHL disease.

Materials and Methods
VHL disease patients treated for spinal cord hemangioblastomas between 1999 and 2009 were included. All spinal cord hemangioblastomas were divided into three groups: Group 1, asymptomatic tumors at initial diagnosis followed with serial imaging studies; Group 2, asymptomatic tumors at initial diagnosis that were subsequently resected; and Group 3, symptomatic tumors at initial diagnosis, all of which were resected.

Results
We identified 24 spinal cord hemangioblastomas in 12 patients. Groups 1, 2 and 3 comprised 13, 4 and 7 tumors, respectively. Group 1 exhibited a smaller tumor volume (257.1 mm3) and syrinx size (0.8 vertebral columns) than those of Group 2 (1304.5 mm3, 3.3 vertebral columns) and Group 3 (1787.4 mm3, 6.1 vertebral columns). No difference in tumor volume or syrinx size was observed between Groups 2 and 3. Five tumors in Group 1 were resected during follow-up because symptoms had developed or the tumor had significantly grown. Finally, among 17 asymptomatic tumors at the initial diagnosis, nine tumors were resected. Only one tumor of these nine tumors resulted in neurological deficits, while five of seven symptomatic tumors caused neurological deficits.

Conclusion
Selective resection of asymptomatic tumors before they cause neurological deficits might bring about better outcomes.

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Available from: Jae Keun Oh, Aug 27, 2014
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    ABSTRACT: Objective To discuss the diagnosis, treatment and long term prognosis of the spinal hemangioblastoma. Methods The clinical data of 33 spinal hemangioblastoma cases, verified by microsurgeries and pathological results, were retrospectively analyzed. Twenty-four of them were males and nine were females. The magnetic resonance imaging (MRI) showed that the 33 cases included 1 medulla-cervical hemangioblastoma, 9 cervical hemangioblastomas, 2 cervical-thoracic hemangioblastomas, 16 thoracic hemangioblastomas, 1 thoracic-lumbar hemangioblastoma, and 4 lumbar hemangioblastomas. Results Total removal of hemangioblastomas was achieved in32 patients and subtotal removal in 1, with no operation associated death. Postoperative follow-up period ranged from 16 months to 12 years. According to the McCormick scale, the functional outcomes were verified as 15(45. 5%) improved, 14(42. 4%) unchanged, and 4(12. 1%) deteriorated. According to the Klekamp-Samn score, the functional outcomes were verified as 24 (72. 7%) improved, 5(15. 2%) unchanged, and 4(12. 1%) deteriorated. Fisher's exact test showed that the pre-operation neuronal function was significantly associated with the prognosis (P = 0. 033). Conclusion Spinal hemangioblastomas are highly vascularized benigntumors, and should be resected early when the symptoms or signs present. Microsurgery is the prior protocol of treatment, and the prognosis after microsurgery is generally fine. Post-operative functional status is determined by pre-operation functional status. Klekamp-Samii score system is more suitable for the functional evaluation of spinal hemangioblastoma.
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