J Korean Neurosurg Soc.  2012 Sep;52(3):221-227.

Surgical Outcome of Spinal Cord Hemangioblastomas

• Park CH
• Lee CH
• Hyun SJ
• Jahng TA
• Kim HJ
• Kim KJ

Abstract

OBJECTIVE
Spinal cord hemangioblastomas are rare tumors. Despite their benign, slow-growing nature, they can cause severe neurological consequences. The purpose of this study was to evaluate variable factors, including clinical features, tumor findings, the extent of resection, and its recurrence or progression, which determine postoperative functional outcomes.
METHODS
This study included sixteen patients at our institute who underwent microsurgical resection for sporadic spinal intramedullary hemangioblastomas and spinal intramedullary hemangioblastomas associated with von Hippel-Lindau (VHL) disease, between June 2003 and March 2012.
RESULTS
A total of 30 operations were performed. Total resection (TR) of the tumor was achieved in 10 patients, and subtotal resection (STR) was achieved in 6. Postoperatively, the initial presenting symptoms were improved in 18.7% of the patients and were unchanged in 56.3%, but 25% were worse. Stable postoperative neurological functions were found in 83% of patients with preoperative McCormick grade I, and TR was achieved in 75% of these patients. In the STR group, poorer neurological status was observed in one patient, despite multiple operations. There were no poorer outcomes in the four cases of VHL disease. Various factors were analyzed, but only a correlation between the pre- and postoperative neurological status was verified in the TR-group patients.
CONCLUSION
Preoperative focal neurological impairment and meticulous microsurgical manipulation may be predictors of favorable outcomes for solitary hemangioblastomas. In addition, the preservation of function is more important than the extent of resection in VHL disease.

Keyword

Hemangioblastoma; Spinal cord; Functional outcome; Von Hippel-Lindau disease

MeSH Terms

Hemangioblastoma
Humans
Recurrence
Spinal Cord
von Hippel-Lindau Disease

Figure

• 

  Fig. 1 Case 5. Preoperative MRI : T2-weighted sagittal image (A) showing a hyperintense lesion with spinal cord swelling from C2 to T1. Gadolinium-enhanced T1 sagittal image (B) revealing a heterogeneous well enhancing mass at the C3-4 spinal cord level. Preoperative angiography (C) showing a hypervascular intramedullary mass at the C3 level fed by the posterior spinal and Lt ascending cervical artery. Postoperative MRI : enhanced T1 sagittal image (D) showing no residual enhanced mass and nearly disappeared cervical cord swelling.

• 

  Fig. 2 Case 16. Gadolinium-enhanced T1 sagittal image (A) showing multiple variably sized, well-enhanced subpial nodules at the first operation site. Sequentially enhanced T1 sagittal image (B) revealing the recurrent lesion at the level of T11. Postoperative enhanced T1 sagittal image (C) showing the presence of small residual nodules. Contrast-enhanced brain sagittal image (D) showing asymptomatic cervical lesions. Image of the tumor increasing in size and becoming symptomatic (E). Postoperative enhanced T1-weighted image (F) showing no residual tumor at the operation site.

Reference

1. Boström A, Hans FJ, Reinacher PC, Krings T, Bürgel U, Gilsbach JM, et al. Intramedullary hemangioblastomas : timing of surgery, microsurgical technique and follow-up in 23 patients. Eur Spine J. 2008; 17:882–886. PMID: 18389290.

2. Cristante L, Herrmann HD. Surgical management of intramedullary hemangioblastoma of the spinal cord. Acta Neurochir (Wien). 1999; 141:333–339. discussion 339-340. PMID: 10352742.

3. Gläsker S, Berlis A, Pagenstecher A, Vougioukas VI, Van Velthoven V. Characterization of hemangioblastomas of spinal nerves. Neurosurgery. 2005; 56:503–509. discussion 503-509. PMID: 15730575.

4. Kanno H, Yamamoto I, Nishikawa R, Matsutani M, Wakabayashi T, Yoshida J, et al. Spinal cord hemangioblastomas in von Hippel-Lindau disease. Spinal Cord. 2009; 47:447–452. PMID: 19030009.

5. Lee DK, Choe WJ, Chung CK, Kim HJ. Spinal cord hemangioblastoma : surgical strategy and clinical outcome. J Neurooncol. 2003; 61:27–34. PMID: 12587793.

6. Lonser RR, Glenn GM, Walther M, Chew EY, Libutti SK, Linehan WM, et al. von Hippel-Lindau disease. Lancet. 2003; 361:2059–2067. PMID: 12814730.

7. Lonser RR, Oldfield EH. Microsurgical resection of spinal cord hemangioblastomas. Neurosurgery. 2005; 57:372–376. discussion 372-376. PMID: 16234688.

8. Lonser RR, Oldfield EH. Spinal cord hemangioblastomas. Neurosurg Clin N Am. 2006; 17:37–44. PMID: 16448906.

9. Lonser RR, Weil RJ, Wanebo JE, DeVroom HL, Oldfield EH. Surgical management of spinal cord hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg. 2003; 98:106–116. PMID: 12546358.

10. Malis LI. Atraumatic bloodless removal of intramedullary hemangioblastomas of the spinal cord. J Neurosurg. 2002; 97:1–6. PMID: 12120629.

11. Mandigo CE, Ogden AT, Angevine PD, McCormick PC. Operative management of spinal hemangioblastoma. Neurosurgery. 2009; 65:1166–1177. PMID: 19934977.

12. McCormick PC, Torres R, Post KD, Stein BM. Intramedullary ependymoma of the spinal cord. J Neurosurg. 1990; 72:523–532. PMID: 2319309.

13. Mehta GU, Asthagiri AR, Bakhtian KD, Auh S, Oldfield EH, Lonser RR. Functional outcome after resection of spinal cord hemangioblastomas associated with von Hippel-Lindau disease. J Neurosurg Spine. 2010; 12:233–242. PMID: 20192620.

14. Na JH, Kim HS, Eoh W, Kim JH, Kim JS, Kim ES. Spinal cord hemangioblastoma : diagnosis and clinical outcome after surgical treatment. J Korean Neurosurg Soc. 2007; 42:436–440. PMID: 19096585.

15. Oppenlander ME, Spetzler RF. Advances in spinal hemangioblastoma surgery. World Neurosurg. 2010; 74:116–117. PMID: 21299999.

16. Pietilä TA, Stendel R, Schilling A, Krznaric I, Brock M. Surgical treatment of spinal hemangioblastomas. Acta Neurochir (Wien). 2000; 142:879–886. PMID: 11086826.

17. Richard S, Campello C, Taillandier L, Parker F, Resche F. French VHL Study Group. Haemangioblastoma of the central nervous system in von Hippel-Lindau disease. J Intern Med. 1998; 243:547–553. PMID: 9681857.

18. Shin DA, Kim SH, Kim KN, Shin HC, Yoon DH. Surgical management of spinal cord haemangioblastoma. Acta Neurochir (Wien). 2008; 150:215–220. discussion 220. PMID: 18253694.

19. Takai K, Taniguchi M, Takahashi H, Usui M, Saito N. Comparative analysis of spinal hemangioblastomas in sporadic disease and Von Hippel-Lindau syndrome. Neurol Med Chir (Tokyo). 2010; 50:560–567. PMID: 20671381.