Yonsei Med J.  2012 Nov;53(6):1073-1080. 10.3349/ymj.2012.53.6.1073.

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

• Kim TY
• Yoon DH
• Shin HC
• Kim KN
• Yi S
• Oh JK
• Ha Y

• KMID: 1414246
• DOI: http://doi.org/10.3349/ymj.2012.53.6.1073

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.

Keyword

Hemangioblastoma; spinal cord; treatment planning; von Hippel-Lindau disease

MeSH Terms

Adult
Aged
Aged, 80 and over
Female
Hemangioblastoma/etiology/*pathology/*surgery
Humans
Male
Middle Aged
Treatment Outcome
von Hippel-Lindau Disease/*complications

Figure

• 

  Fig. 1 Illustration of tumor No.1. (A) Spinal cord hemangioblastoma is seen at L1-2 (arrowed) on contrast-enhanced T1 weighted image. The tumor was asymptomatic and followed with observation. Another tumor at T7-8 and an extensive leptomeningeal enhancement along the spinal cord were also found. (B) The tumor at L1-2 (arrowed) grew almost twice as large after 15 months. The tumor led to rapidly progressive paraparesis, which was not improved after surgery.

• 

  Fig. 2 Illustration of tumor No.2. (A) Initial MRI showed spinal cord hemangioblastoma at T12. The tumor was asymptomatic, with a tumor volume of 150 mm3. (B) 19 months after initial MRI. The tumor increased in volume to 240 mm3. (C) 45 months after initial MRI. The tumor grew to a volume of 468 mm3 and was still asymptomatic. However, it showed steady growth and was regarded as likely to produce neurological symptoms after a short period, so surgical resection was performed. After surgery, no neurological symptoms or deficit developed.

• 

  Fig. 3 Proposed treatment strategy for spinal cord hemangioblastomas in patients with VHL disease. A tumor volume >500 mm3 (10 mm in diameter) is thought to be correlated with symptom formation and functional outcomes, so resection should be performed thereon. *Tumors can be followed at approximately 1-year intervals, if there are no changes in neurological symptoms and size of the tumor. †The mean growth rate of spinal cord hemangioblastomas associated with syringes is reported to be about 50 mm3/month.7 Accordingly, progressively growing tumors larger than a volume of 51 mm3 in combination with syringes are predicted to produce neurological symptoms or deficits within several months to a year. Thus, we recommend considering surgery or intense observation at short intervals of 6 months or less.

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