2020 Edition
| Editors: Maria Rosaria Raspollini, Antonio Lopez-Beltran


  • Alessandro FranchiEmail author
Reference work entry
DOI: https://doi.org/10.1007/978-3-030-41894-6_4821


Capillary hemangioblastoma; Extraneural hemangioblastoma


Hemangioblastoma is a benign tumor of uncertain histogenesis that consists of a proliferation of capillaries accompanied by lipid-containing stromal cells. It occurs sporadically or in association with von Hippel-Lindau disease in approximately 25% of the cases. It arises in the central nervous system in the majority of the cases, but it has been described in several extraneural sites, including the kidney and the bladder.

Clinical Features

  • Incidence

    Hemangioblastoma of the kidney is a rare occurrence, with approximately 15 cases reported.

  • Age

    Patients are mainly young adults.

  • Sex

    Hemangioblastoma of the kidney affects both male and female patients.

  • Site

    Urogenital hemangioblastomas arise mainly in the kidney. Exceptional cases have been reported in the bladder.

  • Treatment

    Surgery is the treatment of choice, and it is often performed with the clinical suspicion of malignancy.

  • Outcome

    Hemangioblastoma is a...

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References and Further Reading

  1. Ip, Y. T., Yuan, J. Q., Cheung, H., et al. (2010). Sporadic hemangioblastoma of the kidney: An underrecognized pseudomalignant tumor? The American Journal of Surgical Pathology, 34, 1695–1700.PubMedGoogle Scholar
  2. Liu, Y., Qiu, X. S., & Wang, E. H. (2012). Sporadic hemangioblastoma of the kidney: A rare renal tumor. Diagnostic Pathology, 7, 49.CrossRefGoogle Scholar
  3. Muscarella, L. A., Bisceglia, M., Galliani, C. A., Zidar, N., Ben-Dor, D. J., Pasquinelli, G., la Torre, A., Sparaneo, A., Fanburg-Smith, J. C., Lamovec, J., Michal, M., & Bacchi, C. E. (2018). Extraneuraxial hemangioblastoma: A clinicopathologic study of 10 cases with molecular analysis of the VHL gene. Pathology, Research and Practice, 214, 1156–1165.CrossRefGoogle Scholar
  4. Wu, Y., Wang, T., Zhang, P. P., Yang, X., Wang, J., & Wang, C. F. (2015). Extraneural hemangioblastoma of the kidney: The challenge for clinicopathological diagnosis. Journal of Clinical Pathology, 68, 1020–1025.CrossRefGoogle Scholar
  5. Zhao, M., Williamson, S. R., Yu, J., Xia, W., Li, C., Zheng, J., Zhu, Y., Sun, K., Wang, Z., & Cheng, L. (2013). PAX8 expression in sporadic hemangioblastoma of the kidney supports a primary renal cell lineage: Implications for differential diagnosis. Human Pathology, 4, 2247–2255.CrossRefGoogle Scholar

Copyright information

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Authors and Affiliations

  1. 1.Department of Translational Research and of New Technologies in Medicine and SurgeryUniversity of PisaPisaItaly