RESUMEN
INTRODUCTION: Renal mucinous tubular and spindle cell carcinoma is a rare subtype of renal cell carcinoma newly added to the World Health Organization classification in 2004. Although it has been considered as a tumor with good prognosis, aggressive cases have recently been reported. CASE PRESENTATION: A 52-year-old man was diagnosed as having left renal cell carcinoma. Open radical left nephrectomy and left-sided pelvic lymph nodes dissection were performed. Pathological diagnosis revealed a renal mucinous tubular and spindle cell carcinoma with high nuclear grade and extra-regional lymph nodes metastasis classified as pT3aN0M1. After nephrectomy, metastasis at second lumbar vertebra and lymph nodes recurrence were occurred. CONCLUSION: This tumor with high nuclear grade may be potentially aggressive and carries a poor prognosis.