Synonyms
Hemangiosarcoma; Malignant hemangioendothelioma
Definition
Angiosarcoma (AS) is a malignant tumor of endothelial cells.
Clinical Features
Incidence
AS of the urogenital tract is very rare, with most studies consisting of single case reports. A weak association with previous radiotherapy has been reported.
Age
It occurs in adult and elderly patients.
Sex
AS of the kidney occurs more frequently in males than females (M:F = 7:1).
Site
Urogenital angiosarcomas arise more frequently in the kidney and prostate. Rare cases have been described in the ureter, the bladder, the epididymis, and the penis.
Treatment
Surgery is the primary treatment, with radiotherapy and chemotherapy employed in approximately 30% of the patients.
Outcome
AS is a high-grade tumor with frequent local relapse and lymph node and distant metastases. The lungs and liver are the most common sites of metastasis.
Macroscopy
Grossly, the tumor presents as an ill-defined, invasive hemorrhagic mass often with areas of...
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References and Further Reading
Abarzua-Cabezas, F., Earle, J., & Meraney, A. (2014). Angiosarcoma of the corpora cavernosa. International Journal of Urology, 21, 622–623.
Brown, J. G., Folpe, A. L., Rao, P., Lazar, A. J., Paner, G. P., Gupta, R., Parakh, R., Cheville, J. C., & Amin, M. B. (2010). Primary vascular tumors and tumor-like lesions of the kidney: A clinicopathologic analysis of 25 cases. The American Journal of Surgical Pathology, 34, 942–949.
Gogoi, D., Hazra, S., Ghosh, B., & Pal, D. (2013). Angiosarcoma of penis. BML Case Reports, 2013. pii: bcr2013200878.
Hayn, M. H., Bastacky, S., & Franks, M. E. (2007). Epididymal angiosarcoma. Urology, 69, 576.e5–576.e7.
Omiyale, A. O., & Carton, J. (2018). Clinical and pathologic features of primary Angiosarcoma of the kidney. Current Urology Reports, 19, 4.
Padaki, P., Hutton, R., Amer, T., Hasan, R., Pugh, D., & Little, B. (2016). A rare case of primary epithelioid angiosarcoma of the ureter. Annals of the Royal College of Surgeons of England, 98, e184–e185.
Seethala, R. R., Gomez, J. A., & Vakar-Lopez, F. (2006). Primary angiosarcoma of the bladder. Archives of Pathology & Laboratory Medicine, 130, 1543–1547.
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Franchi, A. (2020). Angiosarcoma. In: Raspollini, M.R., Lopez-Beltran, A. (eds) Uropathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-41894-6_4780
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DOI: https://doi.org/10.1007/978-3-030-41894-6_4780
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