Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken


  • Alessandro FranchiEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_4780-1



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.


Grossly, the tumor presents as an ill-defined, invasive hemorrhagic mass often with areas of necrosis. AS are often large lesions ranging from 3 to 30 cm in maximum dimension.


Histologically, AS consists of anastomosing vascular channels lined by atypical endothelial cells, often exhibiting multilayering and papillary tufting. Neoplastic cells present a spindle or epithelioid morphology (Fig. 1). Foci of coagulative necrosis, extensive hemorrhage, and brisk mitotic activity are frequently seen.
Fig. 1

Angiosarcoma with solid growth pattern and predominant epithelioid morphology


AS is positive for endothelial cell markers, including CD34, CD31, ERG, FLI1, and factor VIII-related antigen. The lymphatic endothelial marker podoplanin may also be expressed. Epithelioid AS is frequently immunoreactive for cytokeratins.

Molecular Features

Molecular studies have not been conducted so far on urogenital AS. In general, AS presents distinct upregulation of vascular-specific receptor tyrosine kinases and mutations of genes involved in the control of angiogenesis.

Differential Diagnosis

Well-differentiated AS may be difficult to distinguish from anastomosing hemangioma. This benign vascular tumor is composed of anastomosing capillary-sized vessels lined by cytologically bland endothelial cells with hobnail appearance. The complex architecture may resemble that of AS, but it lacks atypical features, including nuclear hyperchromasia, multilayering, atypical mitotic figures, and necrosis.

Poorly differentiated angiosarcomas with solid architecture must be distinguished from other high-grade sarcomas, from sarcomatoid carcinomas and melanoma. Kaposi sarcoma can be excluded based on the absence of HHV8 immunoreactivity.

References and Further Reading

  1. Abarzua-Cabezas, F., Earle, J., & Meraney, A. (2014). Angiosarcoma of the corpora cavernosa. International Journal of Urology, 21, 622–623.CrossRefGoogle Scholar
  2. 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.CrossRefGoogle Scholar
  3. Gogoi, D., Hazra, S., Ghosh, B., & Pal, D. (2013). Angiosarcoma of penis. BML Case Reports, 2013. pii: bcr2013200878.Google Scholar
  4. Hayn, M. H., Bastacky, S., & Franks, M. E. (2007). Epididymal angiosarcoma. Urology, 69, 576.e5–576.e7.CrossRefGoogle Scholar
  5. Omiyale, A. O., & Carton, J. (2018). Clinical and pathologic features of primary Angiosarcoma of the kidney. Current Urology Reports, 19, 4.CrossRefGoogle Scholar
  6. 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.CrossRefGoogle Scholar
  7. Seethala, R. R., Gomez, J. A., & Vakar-Lopez, F. (2006). Primary angiosarcoma of the bladder. Archives of Pathology & Laboratory Medicine, 130, 1543–1547.Google Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

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