Clinicopathological features of translocation renal cell carcinoma

Abstract Background Translocation renal cell carcinomas (TCC) are associated with variable genetic rearrangements of TFE3gene on chromosome Xp11.2. Translocation tumors represent 1% to 5% of all cases of RCC, with the highest frequency among children and young adults. Objectives We sought to characterize clinicopathological features of translocation RCC in Middle Eastern institution. Materials and Methods Clinical and pathological data from a single institution were retrospectively reviewed for 14 patients with translocation RCC between 2005 and 2014. Outcome measures included patient characteristics, clinical manifestations, pathological, treatment outcomes. cancer-specific survival and progression-free survival. Results The mean age at diagnosis was 35 years. Five were females. All were incidentally diagnosed except 2.The mean tumor size was 9 cm, 1 patient had bilateral tumors and 3 presented with positive nodes. All of them had Fuhrman grade 3/4. Three patients underwent partial nephrectomy. Three patients developed metastasis; 4, 5 months and 3 years after diagnosis. One patent died 4 months after surgery and one 21 months after surgery (both due to metastasis). Disease free survival was 71% at mean follow-up of 31 months. Conclusion Translocation RCC is a rare and potentially aggressive subtype of kidney cancer. The overall survival of more than 3 years has been noted unless patient presented with metastasis at time of diagnosis.