Abstract
BACKGROUND: Primary mesenchymal chondrosarcoma of the kidney is an extremely rare malignant tumor. To our best knowledge, only 9 such cases have been reported so far. CASE PRESENTATION: In the current paper, we present the case of a 67 year-old patient with recurrent left lumbar pain, increased fatigability and intermittent macroscopic hematuria. He underwent a surgical resection of the left kidney and left hemicolon.The pathological diagnosis was primary extraskeletal renal mesenchymal chondrosarcoma. Overall survival was 9 months, with pulmonary metastasis and local recurrence at 6 months. The management of the patient is described, from the initial differential diagnosis, after the first clinical examination to the surgical resection, with a special emphasis on the surgical procedures that were carried out. CONCLUSION: Extraskeletal chondrosarcoma of primary origin in the kidney are extremely rare tumors with a highly malignant potential and very poor prognosis. Because the role of chemotherapy or radiation therapy has not been evaluated properly yet, we underline the importance of surgery in the management of such cases as the main and best approach to achieve clinical remission and long-term survival, provided the patient is referred to a surgical consult in time.