Journal of Korean Neurosurgical Society

The Korean Neurosurgical Society (대한신경외과학회)
권호 표지

Treatment Strategy of Multiple Hemangioblastomas

  • Kim, Eui-Hyun (Department of Neurosurgery, Yonsei University College of Medicine) ;
  • Park, Yong-Sook (Department of Neurosurgery, Yonsei University College of Medicine) ;
  • Chang, Jong-Hee (Department of Neurosurgery, Yonsei University College of Medicine) ;
  • Chang, Jin-Woo (Department of Neurosurgery, Yonsei University College of Medicine) ;
  • Park, Yong-Gou (Department of Neurosurgery, Yonsei University College of Medicine)
  • Published : 2005.09.28
Download PDF
⟨ Previous Next ⟩

Abstract

Objective : Hemangioblastomas are highly vascular and benign neoplasm of the central nervous system[CNS]. They can often be found as multiple lesions, as is commonly observed in von Hippel-Lindau[VHL] disease. The aim of this study is to determine the proper management for multiple hemangioblastomas. Methods : Since 1990, 78cases of hemangioblastoma have been encountered. Among these, 9cases were multiple hemangioblastomas that were treated with surgical resection with or without radiosurgery. The medical, radiological, surgical and histological records were reviewed retrospectively and analyzed statistically. Results : Nine patients presented with multiple hemangioblastomas and were diagnosed as VHL disease. The mean follow-up duration was 75.7months [$6.6{\sim}159.2months$] after the first surgical treatment. Three patients were treated with surgical resection alone and six patients were treated by both surgical resection and radiosurgery. Twenty-one surgical procedures [13 surgical resections and 8 radiosurgery] were performed. One patient required ventriculoperitoneal shunt and a posterior fossa decompressive craniectomy because of post-radiation brain swelling. Another patient refused additional treatment for the newly developed lesions after the successful treatment of initial lesions. The other patient who presented with numerous lesions in the whole brain and spine underwent cranio-spinal irradiation. Remaining patients showed good results. Conclusion : The surgical outcomes for the patients with a single lesion of the CNS hemangioblastoma are favorable. However. the treatment of multiple hemangioblastoma is more difficult, and should be treated by surgical resection and radiosurgery with careful consideration.

Keywords

References

  1. 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 43 : 28-34, 1998 https://doi.org/10.1097/00006123-199807000-00018
  2. de la Monte SM, Horowitz SA : Hemangioblastomas : clinical and histopathological factors correlated with recurrence. Neurosurgery 25 : 695-698, 1989 https://doi.org/10.1227/00006123-198911000-00002
  3. Horton WA Wong V, Eldridge R: Von Hippel-Lindau disease: Clinical and pathological manifestation in nine families with 50 affected members. Arch Intern Med 136: 769-777, 1976 https://doi.org/10.1001/archinte.136.7.769
  4. Jawahar A, Kondziolka D, Garces YI, Flickinger JC, Pollock BE, Lunsford LD : Stereotactic radiosurgery for hemangioblastomas of the brain. Acta Neurochir (Wien) 142: 641-644, 2000 https://doi.org/10.1007/s007010070107
  5. Jeffreys R : Clinical and surgical aspects of posterior fossa haeman-gioblastomata. J Neurol Neurosurg Psychiatry 38: 105-111, 1975 https://doi.org/10.1136/jnnp.38.2.105
  6. Ko JH, Park BJ, Kim YJ, Cho MK: LINAC radiosurgery for heman-gioblastoma. J Korean Neurosurg Soc 35 : 529-532, 2004
  7. Lonser RR, Glenn GM, Walther M, Chew EY, Libutti SK, Linehan WM, et al : von Hippel-Lindau disease. Lancet 361 : 2059-2067, 2003 https://doi.org/10.1016/S0140-6736(03)13643-4
  8. Melmon KL, Rosen SW: Lindau's disease. Am J med 36 : 595-617, 1964 https://doi.org/10.1016/0002-9343(64)90107-X
  9. Mohan J, Brownell B, Oppenheimer DR: Malignant spread of haemangioblastoma:report on two cases. J Neurol Neurosurg Psychiatry 39 : 515-525,1976 https://doi.org/10.1136/jnnp.39.6.515
  10. 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 70: 24-30, 1989 https://doi.org/10.3171/jns.1989.70.1.0024
  11. Newmann HP, Lips CJ, Hsia YE, Zbar B: Von Hippel-Lindau syndrome. Brain Pathol 5: 181-193, 1995 https://doi.org/10.1111/j.1750-3639.1995.tb00592.x
  12. Niemela M, Lim YJ, Soderman M, Jaaskelainen J, Lindquist C : Gamma knife radiosurgery in 11 hemangioblastomas. J Neurosurg 85 : 591-596, 1996 https://doi.org/10.3171/jns.1996.85.4.0591
  13. Page KA, Wayson K, Steinberg GK Adler JR: Stereotaxic radiosurgical ablation: an alternative treatment for recurrent and multifocal heman-gioblastomas. A report of four cases. Surg Neurol 40 : 424-428, 1993 https://doi.org/10.1016/0090-3019(93)90225-P
  14. Patrice SJ, Sneed PK, Flickinger JC, Shrieve DC, Pollock BE, Alexander E 3rd, et al : Radiosurgery for hemangioblastorna : results of a multiinstitutional experience. Int J Radiat Oncol Biol Phys 35 : 493-499, 1996 https://doi.org/10.1016/S0360-3016(96)80011-3
  15. Reyns N, Assaker R, Louis E, Lejeune JP : Leptomeningeal hemangioblastomatosis in a case of von Hippel-Lindau disease: case report. Neurosurgery 52: 1212-1216, 2003 https://doi.org/10.1227/01.NEU.0000057838.62257.FE
  16. Smalley SR, Schomberg PJ, Earle JD, Laws ER, Scheithauer BW, O'Fallon JR : Radiotherapeutic considerations in the treatment of hemangioblastomas of the central nervous system. Int J Radiat Oncol Biol Phys 18: 1165-1171, 1990 https://doi.org/10.1016/0360-3016(90)90454-R
  17. Sora S, Ueki K, Saito N, Kawahara N, Shitara N, Kirino T : Incidence of von Hippel-Lindau disease in hemangioblastoma patients: the University of Tokyo Hospital experience from 1954-1998. Acta Neurochir (Wien) 143 : 893-896, 2001 https://doi.org/10.1007/s007010170019
  18. Wanebo JE, Lonser RR, Glenn GM, Oldfieid EH: The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg 98 : 82-94, 2003 https://doi.org/10.3171/jns.2003.98.1.0082
  19. Weil RJ, Vortmeyer AO, Zhuang Z, Pack SD, Theodore N, Erickson RK, et al : Clinical and molecular analysis of disseminated hemangioblastomatosis of the central nervous system in patients without von Hippel-Lindau disease. Report of four cases. J Neurosurg 96: 775-787, 2002 https://doi.org/10.3171/jns.2002.96.4.0775