Summary

Primary renal angiosarcoma is an exceedingly rare and aggressive neoplasm. Although it may occur in youth, this tumour is frequently reported in the sixth and seventh decades of life. The clinical presentation is frequently varied. Pathogenesis remains largely unknown and it has overlapping features with other tumours of the kidney. Current treatment options include variable combinations of surgery, chemotherapy and radiotherapy. Reports regarding the disease prognosis and natural history are limited. In this article, we chronicle the case of a patient with primary renal angiosarcoma presenting at an advanced stage as a widely metastasised tumour. Additionally, we undertake here a brief literature review highlighting the rarity and aggressiveness of this condition, its poor prognosis, and the lack of specific management guidelines.