Treatment of advanced pediatric renal cell carcinoma.

Background: Pediatric renal cell carcinoma (pRCC) is the second most common renal malignancy of childhood; however, treatment data for advanced disease is lacking.
Methods: A retrospective analysis of pRCC patients (age < 21 years at diagnosis) treated between 2000 and 2015 at Cincinnati Children's Hospital Medical Center was undertaken, with specific focus on medical therapies, accompanied by a detailed literature review.
Results: Twenty-four patients (median age = 15 years) were identified; 11 were female. Past history of kidney pathology (4) and prior hematologic/oncologic diagnoses (5) were common associated findings. Translocation morphology RCC (tRCC) was the most common subtype (16; 64%), followed by papillary (6; 24%), clear cell renal cell carcinoma (ccRCC) (1), and chromophobe (1). The TNM stage distribution was I (8; 33%), II (2; 8%), III (3; 13%), and IV (11; 46%). Eleven patients with stage IV disease all had tRCC and received medicinal anticancer therapies, the most common being antiangiogenic (10), conventional chemotherapy (8), mTOR inhibition (7), and immunotherapy (3). Four patients also received small-port radiotherapy. The mean time to progression (TTP) was longest for axitinib (n = 2; TTP = 7.8 m; range 5.5-10 m) and sunitinib (n = 6; TTP = 4.7 m; range 0.3-12 m). Overall, 20 cases of pediatric RCC who received RCC-directed medicinal therapy with outcome data have been previously reported.
Conclusions: For patients with unresectable pRCC requiring systemic therapy, available data are scarce. Data herein support an increased TTP with antiangiogenic therapy in tRCC supporting a formal study of antiangiogenic therapies through multicooperative-group collaboration.
(© 2019 Wiley Periodicals, Inc.)