Hemangioblastoma and its treatment using Leksell Gamma Knife

Authors: J. Valchář;  R. Liščák;  G. Šimonová;  J. Vymazal
Authors‘ workplace: Oddělení stereotaktické a radiační neurochirurgie, Nemocnice Na Homolce, Praha
Published in: Cesk Slov Neurol N 2008; 71/104(2): 216-222
Category: Short Communication


Between 1993 and 2006, 15 hemangioblastoma patients were treated at our department with the use of stereotactic radiosurgery. The group consisted of 8 men and 7 women aged between 14 and 72 years (mean age being 39 years). In 9 patients hemangioblastomas were associated with von Hippel-Lindau disease; in 6 patients sporadic hemangioblastomas were diagnosed. Solitary hemangioblastomas were detected in 6 multilocular in 9 patients, respectively. A total of 43 hemangioblastomas with a diameter ranging from 2 to 30 mm (the mean diameter being 10.7 mm) were irradiated. The mean tumour margin dose was 18.8 Gy (ranging from 9 to 22 Gy, the median being 20 Gy), the maximum dose into the tumour centre was 35.4 Gy (ranging from 20 to 44 Gy, the median being 36). For 13 patients, post-op follow-up results were obtained within 11 to 120 months (with the mean of 55 months and the median of 48 months). Stabilisation or improvement of clinical status was recorded for 10 patients, progression occurred in 3 patients (23 %). Among the irradiated tumours, 11 regressed and 14 showed no change in diameter, i.e. local control was attained in 80.6 % of tumours (25 out of the 31 controlled tumours). 6 irradiated tumours progressed (of which 2 due to solid part progression and 4 due to cystic part progression: stereotactic punction was performed in 3 and open neurosurgery in 1 of the 4 tumours, respectively). One patient died of extracranial organ complications associated with von Hippel-Lindau disease. Radiosurgical treatment of hemangioblastomas proved effective in patients with both solitary and multiloculary small or middle size hemangioblastomas, with a good clinical response and a low risk of complications.

Key words:
hemangioblastoma – von Hippel-Lindau disease – Leksell Gamma Knife – radiosurgery


1. Jeffreys R. Clinical and surgical aspects of posterior fossa haemangioblastoma. J Neurol Neurosurg Psychiatry 1975; 38: 105–111.

2. Melmon KL, Rosen SW. Lindau's disease. Review of the literature and study of a large kindred. Am J Med 1964; 36: 595–617.

3. Wanebo JE, Lonser RR, Glenn GM, Oldfield EH. The natural history of hemangioblastomas of the central nervous system in patients with von Hippel–Lindau disease. J Neurosurg 2003; 98: 82–94.

4. Neumann HPH, Berger DP, Sigmund G, Blum U, Schmidt D, Parmer RJ et al. Phaeochromocytomas, multiple endocrine neoplasia type 2, and von Hippel-Lindau disease. N Engl J Med 1993; 329: 1531–1538.

5. Křepelová A. Dědičné formy nádorů ledvin – spektrum predispozičních genů a jejich testování. Klinická onkologie 2006; 19(Suppl): 82–84.

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

7. Richard S, David P, Marsot-Dupuch K, Giraud S, Béroud C, Resche F. Central nervous system hemangioblastomas, endolymphatic sac tumors, and von Hippel–Lindau disease. Neurosurg Rev 2000; 23: 1–22.

8. Smrcka M, Smrcka V. Central nervous system hemangioblastoma: its role in von Hippel-Lindau disease. Bratisl Lek listy 2000; 101: 503–506.

9. Kozler P, Kramář F. Von Hippelova-Lindauova choroba a kapilární hemangioblastom. In: Kozler P et al. Intrakraniální nádory. Praha: Galén 2007: 177–179.

10. Catapano D, Muscarella LA, Guarnieri V, Zelante L, D'Angelo VA, D'Agruma L. Hemangioblastomas of central nervous system: molecular genetic analysis and clinical management. Neurosurg 2005; 56: 1215–1221.

11. Neumann HP, Eggert HR, Weigel K, Friedburg H, Wiestler OD, Schollmeyer P. Hemangioblastomas of the central nervous system. A 10-year study with special reference to von Hippel-Lindau syndrome. J Neurosurg 1989; 70: 24–30.

12. Chakraborti PR, Chakrabarti KB, Doughty D, Plowman PN. Stereotactic multiple are therapy. IV-Haemangioblastoma. Br J Neurosurg 1997; 11: 110–115.

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

14. Slavin KV. Hemangioblastoma. eMedicine Specialties [online]; last revision 13th of April 2006. Dostupné z <http://www.emedicine.com/med/topic2991.htm>.

15. Filling-Katz MR, Choyke PL, Patronas NJ, Gorin MB, Barba D, Chang R et al. Radiologic screening for von Hippel-Lindau disease: the role of Gd-DTPA enhancened MR imaging of the CNS. J Comput Assist Tomogr 1989; 13: 743–755.

16. Vymazal J. Ostatní nádory mozkových obalů. In: Vymazal J. Magnetická rezonance nervové soustavy. Radiologické a klinické aspekty. I. Mozkové nádory. Praha: M-DIAG Publishing 2006: 109–112.

17. 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: 168–171.

18. Eskridge JM, McAuliffe W, Harris B, Kim DK, Scott J, Winn HR. Preoperative endovascular embolization of craniospinal hemangioblastomas. AJNR Am J Neuroradiol 1996; 17: 525– 531.

19. Takeuchi S, Tanaka R, Fujii Y, Abe H, Ito Y. Surgical treatment of hemangioblastomas with presurgical endovascular embolization. Neurol Med Chir (Tokyo) 2001; 41: 246–251.

20. Gläsker S, Van Velthoven V. Risk of hemorrhage in hemangioblastomas of the central nervous system. Neurosurgery 2005; 57: 71–76.

21. Rajaraman C, Rowe JG, Walton L, Malik I, Radatz M, Kemeny AA. Treatment options for von Hippel-Lindau's haemangioblastomatosis: the role of gamma knife stereotactic radiosurgery. Br J Neurosurg 2004; 18: 338–342.

22. Georg AE, Lunsford LD, Kondziolka D, Flickinger JC, Maitz A. Hemangioblastoma of the posterior fossa. The role of multimodality treatment. Arq Neuropsiquiatr 1997; 55: 278–286.

23. 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: 238–243.

24. Jawahar A, Kondziolka D, Garces YI, Flickinger JC, Pollock BE, Lunsford LD. Stereotactic radiosurgery for hemangioblastoma of the brain. Acta Neurochirurg (Wien) 2000; 142: 641–645.

25. Wang EM, Wang BJ, Zhang N, Pan L, Dong YF, Zhou LF et al. Analysis of the results of gamma knife radiosurgery for hemangioblastomas of the brain and the factors related to the tumor recurrence. Zhonghua Yi Xue Za Zhi 2004; 84: 813–817.

26. Park YS, Chang JH, Chang JW, Chung SS, Park G. Gamma knife surgery for multiple hemangioblastomas. J Neurosurg 2005; 102(Suppl): 97–101.

27. Niemelä M, Lim YJ, Söderman M, Jääskeläinen J, Lindquist C. Gamma knife radiosurgery in 11 hemangioblastomas. J Neurosurg 1996; 85: 591–596.

28. Patrice SJ, Sneed PK, Flickinger JC, Shrieve DC, Pollock BE, Alexander E et al. Radiosurgery for hemangioblastoma: results of a multiinstitutional experience. Int J Radiat Oncol Biol Phys 1996; 35: 493–499.

29. Chang SD, Meisel JA, Hancock SL, Martin DP, McManus M, Adler JR jr. Treatment of hemangioblastomas in von Hippel-Lindau disease with linear accelerator-based radiosurgery. Neurosurgery 1998; 43: 28–34.

30. Conway JE, Chou D, Clatterbuck RE, Brem H, Long DM, Rigamonti D Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease. Neurosurgery 2001; 48: 55–62.

31. Schuch G, de Wit M, Höltje J, Laack E, Schilling G, Hossfeld DK et al. Case 2. Hemangioblastomas: diagnosis of von Hippel-Lindau disease and antiangiogenic treatment with SU5416. J Clin Oncol 2005; 23: 3624–3626.

Paediatric neurology Neurosurgery Neurology
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