BACKGROUND:Pediatric non-Wilms' renal tumors (NWRT) are poorly understood owing to their heterogeneity and relative rarity.
This study aimed at auditing the outcome of the management of NWRT in a tertiary hospital in the Third World.
METHODS: Records of all patients (n = 68) treated for NWRT over a 32-year period (1978-2010) were reviewed retrospectively.
RESULTS: The major histological groups included clear cell sarcoma of the kidney (CCSK) (33.8%), mesoblastic nephroma (17.6%), cystic partially differentiated nephroblastoma (CPDN) (17.6%), intrarenal neuroblastoma (8.8%), malignant rhabdoid tumor (MRT) (7.4%), and renal cell carcinoma (RCC) (5.9%). Sixteen (69.7%) patients with CCSK and 11 (91.7%) with CPDN were aged 1-4 years. Ten (83.3%) patients with mesoblastic nephroma were aged < 1 year and three (60.0%) with RCC were aged 10-14 years. Ten (43.5%) patients with CCSK and four (80.0%) with RCC had metastases at diagnosis. The sensitivity of a pretreatment Tru-Cut biopsy was 100% for MRT. All the patients with CCSK, mesoblastic nephroma, CPDN, and RCC had radical nephrectomy. Only eight (34.8%) patients with CCSK received radiotherapy. The overall 1-10-year survival rates were 52.2%, 91.7%, 75.0%, 40.0% and 0.0% for CCSK, mesoblastic nephroma, CPDN, RCC, and MRT, respectively. The overall 1-10-year survival for the entire cohort was 51.5%.
CONCLUSIONS: The demography and clinical presentation of pediatric NWRT, which comprises 13.6% of pediatric renal tumors in the Third World, were similar to those in the Developed World. The overall 1-10-year survival for pediatric NWRT was low.
Written by:
Saula PW, Hadley GP. Are you the author?
Department of Paediatric Surgery, Nelson R. Mandela School of Medicine, University of KwaZulu-Natal, Private Bag 7, Congella 4013, South Africa.
Reference: World J Surg. 2012 Mar;36(3):565-72.
doi: 10.1007/s00268-011-1410-2
PubMed Abstract
PMID: 22270981
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