Renal leiomyosarcoma associated with xeroderma pigmentosum

We report a case of leiomyosarcoma of the kidney, presenting in the course of xeroderma pigmentosum, which debuted clinically as Wünderlich's syndrome due to spontaneous rupture of the tumor. We describe the case history and discuss the cutaneous disorder, analyzing the clinical and etiopathogenic factors, and conclude with an attempt to establish a relationship between the two pathologies.