Skip to main content
  • Supratentorial Hemangioblastomas Without Von-Hippel Lindau Disease

    Final Number:

    Jose Carlos Rodrigues MD; VITHOR E. B. DA SILVA MD; Marcelo Campos; Eberval G. Figueiredo MD, PhD; Renan Lapate; Giana F Kuhn MD; Roberto Bezerra Vital MD

    Study Design:
    Clinical Research

    Subject Category:
    Tumor: Extra-Axial

    Meeting: Congress of Neurological Surgeons 2019 Annual Meeting

    Introduction: Hemangioblastomas are tumors constantly present in the neurosurgeon's daily life, affecting mainly the posterior fossa region. In patients with von Hippel-Lindau, their presence is even more striking. However, the authors, through a report of a case with the literature review, show that hemangioblastomas should also be part of the range of differential diagnoses of supratentorial lesions even in patients not carrying the genetic mutation.

    Methods: We retrospective analyse pertinent literature about supratentorial hemangioblastomas with or without von Hippel disease and illustrate with a case operated by the authors.

    Results: Supratentorial hemangioblastomas are rare with little information in the literature to guide management specially without von Hippel disease. According to this it diagnosis is not suggested until the pathological definition. But as described it must be between the differential diagnosis of extra-axial tumors to accurately make its surgery the best of what is possible to avoid recurrence and had better results. Our case illustrate that with gross total resection , supratentorial hemangioblastoma can be well controlled, with no recurrence even 3 years after surgery.

    Conclusions: Hemangioblastomas are tumors affecting mainly the posterior fossa region specially in patients with von Hippel-Lindau disease. However, the authors, through a report of a case and literature review, show that hemangioblastomas should also be part of the range of differential diagnoses of supratentorial lesions even in patients not carrying the genetic mutation.

    Patient Care: With this research, doctors will could improve care of patients with supratentorial hemangioblastomas, specially concerning on their diagnosis, best strategies to treat and recurrence avoidance.

    Learning Objectives: By the conclusion of this presentation, participants should be able to know natural history of supratentorial hemangioblastomas with or without Von Hippel-Lindau disease, its management and best strategies of treatment. And with the case reported to know and fix special characteristics of this kind of tumors.

    References: 1. Kansu T, Bower M, Waxman J, Frassanito P, Markogiannakis G, Di Bonaventura R, et al. World Health Organization Classification of Tumours of the Central Nervous System [Internet]. Vol. 16, SADJ?: journal of the South African Dental Association = tydskrif van die Suid-Afrikaanse Tandheelkundige Vereniging. 2007. 13-66 p. Available from: 2. Yang B, Luan S, Cao X, Bao W. Supratentorial hemangioblastoma. Neurosciences. 2011;16(2):150–2. 3. Kumar V, Pandey S, Kumar M, Sharma V, Pandey D. Supratentorial haemangioblastoma without von Hippel–Lindau syndrome in an adult: A rare tumor with review of literature. Asian J Neurosurg. 2015;11(1):8. 4. Wenting J, Ogawa Y, Ito J, Tominaga T. Suprasellar Hemangioblastoma Unrelated to von Hippel-Lindau Disease Successfully Treated through Extended Transsphenoidal Approach: Diagnostic Value of Von Hippel-Lindau Disease Gene-Derived Protein. J Neurol Surgery, Part A Cent Eur Neurosurg. 2017;78(3):296–301. 5. Franco A, Pytel P, Lukas R V., Chennamaneni R, Collins JM. CNS hemangioblastomatosis in a patient without von Hippel-Lindau disease. CNS Oncol. 2017;6(2):101–5. 6. Mills SA, Oh MC, Rutkowski MJ, Sughrue ME, Barani IJ, Parsa AT. Supratentorial hemangioblastoma: Clinical features, prognosis, and predictive value of location for von HippelLindau disease. Neuro Oncol. 2012;14(8):1097–104. 7. Lee CY, Chen SJ. Supratentorial leptomeningeal hemangioblastoma resection after tumor embolization. Formos J Surg [Internet]. 2016;49(2):78–81. Available from: 8. Rocha L, Noronha C, Taipa R, Reis J, Gomes M, Carvalho E. Supratentorial hemangioblastomas in von Hippel–Lindau wild-type patients–case series and literature review. Int J Neurosci. 2018;128(3):295–303. 9. Amin-Hanjani S, Valyi-Nagy T, Kord Valeshabad A, Xiao L, Alsadi A, Kim J. Sporadic Hemangioblastoma of the Cavernous Sinus and Meckel’s Cave. J Neurol Surg Reports. 2018;79(04):e98–102. 10. She DJ, Xing Z, Liu Y, Cao DR. Supratentorial hemangioblastomas: Three case reports and review of the literature. Clin Neuroradiol. 2013;23(3):243–7. 11. Zhuang Z, Huntoon K, Lonser RR, Linehan WM, Oldfield EH, Chew EY, et al. Prospective natural history study of central nervous system hemangioblastomas in von Hippel-Lindau disease. J Neurosurg. 2014;120(May):1055–62. 12. Report AC. A Case Report and Review of the Literature. 2003;1–3. 13. French N C I Network V H L Disease and Inherited Predisposition to Kidney Cancer. Natural History of Supratentorial Hemangioblastomas in von Hippel-Lindau Disease. Neurosurgery [Internet]. 2010 Sep 1;67(3):577–87. Available from:

We use cookies to improve the performance of our site, to analyze the traffic to our site, and to personalize your experience of the site. You can control cookies through your browser settings. Please find more information on the cookies used on our site. Privacy Policy