Pediatric non-Wilms' renal tumors: a third world experience.

Abstract

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.

• Authors: Peter W Saula, G P Hadley
• Journal: World journal of surgery (World J Surg) Vol. 36 Issue 3 Pg. 565-72 (Mar 2012) ISSN: 1432-2323 [Electronic] United States
• PMID: 22270981 (Publication Type: Journal Article)

Topics

• Adolescent
• Carcinoma, Renal Cell (surgery)
• Chemotherapy, Adjuvant
• Child
• Child, Preschool
• Humans
• Infant
• Kidney Neoplasms (mortality, surgery, therapy)
• Neoadjuvant Therapy
• Nephroma, Mesoblastic (surgery)
• Retrospective Studies
• Sarcoma, Clear Cell (surgery)
• Wilms Tumor (surgery)