Patterns of management of translocation renal cell carcinoma

Translocation renal cell carcinoma (TRCC) represents 1% to 5% of all cases of renal cell carcinoma (RCC), with the highest frequency among children and young adults. Management of these tumors is ill defined. We sought to characterize clinicopathological features of TRCC and patterns of medical and surgical management in a middle eastern health institute.

Clinical and pathological data of 23 patients from a single institution diagnosed with TRCC between January 2005 and July 2017 were retrospectively reviewed. We dichotomized patients based on demographics, methods of surgical approach and pathologic tumor stage. We then evaluated the methods of medical management for metastatic disease and response to treatment based on cancer-specific survival (CSS) and progression-free survival (PFS).

The median age at diagnosis was 37 years. Fifteen (65%) patients were male. Most of the patients were diagnosed incidentally (65%) during abdominal imaging for other reasons. The mean tumor size was 9 cm, 47% of the patients had pathologic ≥ T3 stage. Eleven patients had lymph node dissection for clinically enlarged lymph nodes, 7 of which (64%) had lymph node metastasis. Partial nephrectomies were performed for three tumors. Eight patients had metastasis (34.7%), and 3 of them had metastasis at the time of diagnosis. Six patients received sunitinib for the treatment of metastatic disease, one patient had complete response, 4 patients had stable disease and one had disease progression. Three patients died during follow-up period because of development of metastasis at postoperative 4 (n=1), and 21 (n=1) months, and cerebral hemorrhage (n=1). The mean follow-up period was 35 months and 3-year disease-free survival was 75%.

TRCC is rarely seen but carries significant risk of disease progression with potential response to targeted therapy.