105 cases of renal tumors in patients age 15 to 35: A specific entity? Experience of the Gustave Roussy Institute.

Abstract

4575 Background: To describe the specific features of malignant renal tumors occurring in young adults. Methods: Medical charts of all 15-35 yo pts diagnosed with a primary renal malignant tumor from1990 to date and treated at Gustave Roussy were retrieved. Results: Overall, 105 pts were analyzed with a median follow up of 21.1 months (IQR 5.53-47.2). First pathological entity was Renal Cell Carcinoma (RCC) (n= 89) and second one was nephroblastoma (n=9). Median age at diagnosis was 28.7(24-32) : 31.1 (27.8-33.6) for RCC and 23.6 (22.2-25.8) for nephroblastoma. Only 24% pts underwent a tumor biopsy as first procedure while others had nephrectomy or tumorectomy front line. Among nephroblastoma, only 2 had first biopsy and received neo-adjuvant chemotherapy. Four had synchronous metastasis, 3 remained localized and received adjuvant chemotherapy and 2 developed peritoneal immediate recurrence after surgery requiring additional chemotherapy and radiation therapy. Regarding ccRCC (37pts), 87% of pts presented with symptoms; 76% presented metastasis at diagnosis or during follow up. Prognosis groups of mRCC (83pts), according to IMDC, were good/intermediate and poor in 44/34/5 respectively. One out of 9 pts with nephroblastoma has died. Conclusions: We report a large cohort of 15-35 yo patients with malignant renal tumors, highlighting (i) the issue of highly aggressive RCC disease in young adults and (ii) the challenge of differential diagnosis of nephroblastoma. Interestingly, nephroblastoma were diagnosed in pts aged up to 31 and diagnosis procedure did not comply with required pediatric guidelines. This report suggests the need of standardized guidelines in young adult renal tumors management. Age [15-20] [20-25] [25-30] [30-35] 1/ Histology Nb of pts Total =105 13 19 28 45 RCC - ccRCC - translocation - pRCC - other RCC Nephroblastoma Miscellaneous 89 37 20 17 15 9 7 10 2 3 2 3 1 2 11 4 3 1 3 4 4 25 10 9 2 4 2 1 43 21 5 12 5 2 0 2/Survival mRCC (n=83) OS (mos) PFS first-line (mos) ccRCC 21.5 (4.6-44.4) 3.2 (1.8-7) translocation 11.1 (5.4-23.4) 3.6 (2.6-6.5) pRCC 19.1 (11-50.6) 2.9 (2.3-9.2)