Background: Leiomyosarcoma represents a rather uncommon malignancy, and reports of cases characterized by a renal genesis are particularly rare.
Cases: We describe 2 cases of leiomyosarcoma of the kidney, in a 63-year-old woman and in a 53-year-old man, respectively. Both tumors share a common immunohistochemical profile with a strong positivity for smooth muscle actin, a focal positivity for desmin, and negativity for cytokeratins and other markers.
Conclusion: We provide a comparison between our findings and the data available in the literature, and we note an interesting relatively long survival in our patients (10 months for the first case and 20 months for the second case).