Purpose of Review
Renal cell carcinoma (RCC) is the most common renal malignancy in adults, accounting for nearly 90% of adult renal tumors. Pediatric RCC has a unique genomic profile and clinical behavior distinct from adult RCC. About 50% of pediatric renal cell carcinomas involve translocation of chromosomal foci which are biologically different from the prominent adult RCC. Given the paucity of cases in adult urology, there may be an opportunity to collaborate and share management strategies and surveillance of translocation RCC by learning from cases in children and adolescents. The aim of this review article is to synthesize the current evidence of adult and pediatric translocation RCC and provide management strategies for translocation RCC.
Recent Findings
There are several key points present in the literature of pediatric translocation RCC that may provide more insight in the management of adult translocation RCC. The unique genomic profile of translocation RCC provides distinct features that differentiate this from other subtypes of RCC. In adult cases, translocation RCC is rarely described accurately on pathology given the lack of integration of genomic arrays and staining that is more frequently utilized in pediatric cases. Lymph node dissection at the time nephrectomy for patients with translocation RCC inaccurately determines pathologic staging and potentially impacts survival. Further investigation is necessary to better understand the survival and progression-free rate in high-risk translocation RCC. Clinical trials (AREN1721) and recruitment efforts are ongoing to understand the benefit of immunotherapy in translocation tumors in both adult and pediatric cases.
Summary
Compared to other subtypes of RCC, translocation tumors are an aggressive malignancy presenting with high-risk features, particularly in children and adolescents. Given the paucity of cases, optimal management of these tumors in children continues to be unclear and efforts are being made to enroll patients onto clinical trials to better understand treatment paradigms. This review summarizes the current state of treatment, diagnosis of translocation RCC, and future endeavors systemic therapy for this aggressive disease. Although there are several gaps in our knowledge about the management of advanced translocation tumors, there are integral aspects of this disease that could help adult clinicians diagnose and treat this subtype of RCC.
