Synonyms
Capillary hemangioblastoma; Extraneural hemangioblastoma
Definition
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
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.
Liu, Y., Qiu, X. S., & Wang, E. H. (2012). Sporadic hemangioblastoma of the kidney: A rare renal tumor. Diagnostic Pathology, 7, 49.
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.
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.
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.
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Franchi, A. (2020). Hemangioblastoma. In: Raspollini, M.R., Lopez-Beltran, A. (eds) Uropathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-41894-6_4821
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DOI: https://doi.org/10.1007/978-3-030-41894-6_4821
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