Abstract
Mesenchymal chondrosarcoma of the kidney is a very rare entity with no definite treatment protocol. Herein, we describe one such case with discussion of its diagnosis and management. The patient had a well circumscribed mass in right kidney extending into the inferior vena cava and metastasis to both the lungs. Right nephrectomy was performed and the histopathological examination confirmed the diagnosis to be renal mesenchymal chondrosarcoma. After surgical removal of the tumor, the patient was given chemotherapy with Cisplatin and Epirubicin, following which there was significant regression of lung nodules.