Skip to main content

Advertisement

SpringerLink
Log in

Surgical management of haemangioblastoma of the posterior fossa

  • Clinical Articles
  • Published:
Acta Neurochirurgica Aims and scope Submit manuscript

Summary

Fifty-one cases of posterior fossa haemangioblastoma have been seen in our department over a period of 23 years. Seven cases were associated with spinal cord lesions and one with a supratentorial lesion.

There were 22 cases of Lindau's disease (43%). The onset was earlier and recurrences commoner than in isolated cases. In 10 cases the development of new tumours in different parts of the central nervous system (CNS) required re-operation, demonstrating the multicentric nature of this disease.

The majority of the tumours occupying the fourth ventricle or medulla oblongata (8 cases) were macroscopically solid and had higher post-operative morbidity than the cystic tumours in the cerebellum.

The recent development of magnetic resonance imaging (MRI) provides accurate anatomical information of the lesions (especially those of the brain stem and spinal cord) even while asymptomatic. The brilliant enhancement of these tumours on MRI with Gadolinium-diethyline-triamine-pentaacetic acid (Gd-DTPA) has proved to be particularly useful.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Carpenter CT, Schwartz H, Walker AE, (1943) Neurogenic polycythaemia. Ann Int Med 19: 470–471

    Google Scholar 

  2. Cornell SM, Hibri NS, Menenez AK, Graf C (1979) The complementary nature of computed tomography and angiography in the diagnosis of cerebellar haemangioblastoma. Neuroradiology 17: 201–205

    Article  PubMed  Google Scholar 

  3. Cushing H, Bailey P (1932) Tumours arising from the bloodvessels of the brain: angiomatous malformations and haemangioblastomas. Thomas, Springfield, Ill

    Google Scholar 

  4. Diehl PR, Symon L (1981) Supratentorial intraventricular haemangioblastoma: case report and review of literature. Surg Neurol 15: 435–443

    PubMed  Google Scholar 

  5. Djindjian M (1986) Successful removal of a brainstem haemangioblastoma. Surg Neurol 25: 97–100

    Article  PubMed  Google Scholar 

  6. Ferrante L, Celli P, Fraioli B, Santoro A (1984) Haemangioblastoma of the posterior cranial fossa. Acta Neurochir (Wien) 71: 283–294

    Article  Google Scholar 

  7. Grossman M, Melmon KL (1972) Von Hippel-Lindau disease. In: Vinken PJ, Bruyn GW (eds) Handbook of clinical neurology: the phakomatoses, Vol 14. North-Holland Publishing, Amsterdam, pp 241–259

    Google Scholar 

  8. Hennessy TG, Stern WE, Kerrick SE (1967) Cerebellar haemangioblastoma. Erythropoietin activity by radiation assay. J Nuclear Med 8: 601–606

    Google Scholar 

  9. Horton WA, Wong V, Eldridge R (1976) Von Hippel-Lindau disease: clinical and pathological manifestations in nine families with 50 affected members. Arch Intern Med 136: 769–777

    Article  PubMed  Google Scholar 

  10. Huson SM, Harper PS, Hourihan MD, Cole G, Weeks RD, Compston DAS (1986) Cerebellar haemangioblastoma and von Hippel-Lindau disease. Brain 109: 1297–1310

    PubMed  Google Scholar 

  11. Ismail SM, Cole G (1984) Von Hippel-Lindau syndrome with microscopic haemangioblastomas of the spinal nerve roots. Case report. J Neurosurg 60: 1279–1281

    PubMed  Google Scholar 

  12. Jackson JH (1872) A series of cases illustrative of cerebral pathology. Cerebral tumour. Medical Times and Gazette, London 2: 541–544

    Google Scholar 

  13. Jeffreys R (1975) Clinical and surgical aspects of posterior fossa haemangioblastomata. J Neurol Neurosurg Psychiatry 38: 105–111

    PubMed  Google Scholar 

  14. Jeffries RV, Napier S, Reynolds SK (1982) Erythropoietin levels in posterior fossa haemangioblastoma. J Neurol Neurosurg Psychiatry 45: 264–266

    PubMed  Google Scholar 

  15. Kamitani H, Masuzawa M, Sato J, Kanazawa I (1987) Eryth-ropoietin in haemangioblastoma. Immuno-histochemical and electron microscopic studies. Acta Neurochir (Wien) 85: 56–62

    Article  Google Scholar 

  16. Levin PM (1936) Multiple hereditary haemangioblastomas of the nervous system. Arch Neurol Psychiatry 36: 384–391

    Google Scholar 

  17. Lindau A (1926) Studien über Kleinhirncysten. Bau, Pathogenese und Beziehungen zur Angiomatosis retinae. APMIS [Suppl] 1: 1–128

    Google Scholar 

  18. Lindau A (1930) Discussion on vascular tumours of the brain stem and spinal cord. Proc Soc Med 24: 363–370

    Google Scholar 

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

    Article  PubMed  Google Scholar 

  20. Miller MH, Tucker WS, Bilbao JM (1986) Supratentorial haemangioblastoma associated with von Hippel-Lindau disease: case report and review of the literature. J Can Assoc Radiol 37: 35–37

    Google Scholar 

  21. Mondkar VP, McKissock W, Ross Russell RW (1967) Cerebellar haemangioblastoma. B J Surg 54: 45–49

    Google Scholar 

  22. Mortelmans MG, Dupont MG (1978) CT scanning for the diagnosis of the cerebellar and spinal lesions of von Hippel-Lindau disease. Neuroradiology 14: 241–244

    Article  PubMed  Google Scholar 

  23. Murota T, Symon L (1989) Surgical management of haemangioblastoma of the spinal cord: a report of 18 cases. Neurosurgery 25(5): 699–708

    Article  PubMed  Google Scholar 

  24. Naidich TP, Lin JP, Leeds NE, Pudlowski RM, Naidich JB (1977) Primary tumours and other masses of the cerebellum and fourth ventricle: differential diagnosis by computed tomography. Neuroradiology 14: 152–174

    Article  Google Scholar 

  25. Neumann HPH, Eggert HR, Weigel K, Friedburg H, Wiestier OD, Schollmeyer P (1989) Haemangioblastomas of the central nervous system. J Neurosurg 70: 24–30

    PubMed  Google Scholar 

  26. Obrador S, Martin-Rodoriguez JG (1977) Biological factors involved in the clinical features and surgical management of cerebellar haemangioblastomas. Surg Neurol 7: 79–84

    PubMed  Google Scholar 

  27. Olivecrona H (1952) The cerebellar angioreticulomas. J Neurosurg 9: 317–330

    PubMed  Google Scholar 

  28. Palmer JJ (1972) Haemangioblastomas. A review of 81 cases. Acta Neurochir (Wien) 27: 125–148

    Article  Google Scholar 

  29. Panas F, Remy A (1879) Anatomie Pathologique de l'Oeil. Delahaye and Cie, Paris, pp 88–98

    Google Scholar 

  30. Robinson RG (1965) Aspects of the natural history of cerebellar haemangioblastomas. Acta Neurol Scand 41: 372–380

    Google Scholar 

  31. Rosenlof IK, Fyhrquist F, Gronhagen-Riska C, Bohling T, Haltia M (1985) Erythropoientin and renin substrate in cerebellar haemangioblastoma. Acta Med Scand 218: 418

    Google Scholar 

  32. Schuback A (1927) Über die Angiomatosis des Zentralnervensystems (Lindausche Krankheit). Ztschr Ges Neurol Psychiat 110: 359–62

    Google Scholar 

  33. Silver ML, Hennigar O (1952) Cerebellar haemangioma: (haemangio-blastomas) clinicopathological review of 40 cases. J Neurosurg 9: 484–494

    PubMed  Google Scholar 

  34. Stack JP, Antoun NM, Jenkins JRP, Metcalfe R, Isherwood I (1988) Gadolinium-DTPA as a contrast agent in magnetic resonance imaging of the brain. Neuroradiology 30: 145–154

    Article  PubMed  Google Scholar 

  35. Tachibana O, Yamashima T, Yamashita J (1991) Immuno-histochemical study of erythropoietin in cerebellar haemangio-blastoma associated with secondary polychythaemia. Neurosurgery 28: 24–26

    Article  PubMed  Google Scholar 

  36. von Hippel E (1904) Über eine sehr seltene Erkrankung der Netzhaut. Albrecht von Graefes Arch Ophthalmol 59: 83–87

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Gough-Cooper Department of Neurological Surgery, The National Hospital for Neurology and Neurosurgery, Queen Square, London, UK

    L. Symon, T. Murota, M. Pell & L. Bordi

Authors
  1. L. Symon
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. T. Murota
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. M. Pell
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. L. Bordi
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Symon, L., Murota, T., Pell, M. et al. Surgical management of haemangioblastoma of the posterior fossa. Acta neurochir 120, 103–110 (1993). https://doi.org/10.1007/BF02112026

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02112026

Keywords

Search

Navigation