Title : Recent advances in pediatric renal tumors
Abstract:
In the pediatric age group, Wilms Tumor (Nephro blastoma), is the commonest (85%). The others being Congenital Mesoblastic Nephroma, Clear Cell Sarcoma and Rhabdoid Tumor.
Wilms Tumor is a Malignant Embryonal Tumor arising from the Nephrogenic blastema and has 3 components blastema, epithelial and stromal component,(triphasic), sometime it is biphasic and monophasic. The prognosis and management is done by 2 systems.
- SIOP (International Society of Pediatric Oncology) and
- COG (Children Oncology Group)
The Genetic markers are WT1 gene at chromosome 11p13, WT2 gene at chromosome 11p15, PAX6, Familial WT1, Familial WT2 and LOH (Loss of Heterozygosity) The Pathological features, like Gross, Microscopy, Clinical features etc will be discussed.
The prognosis depends on the Tumor stage at Diagnosis, Histology, Favourable verses unfavourable histology (diffuse anaplasia), age, LOH at chromosome 1p and 16q.
The other tumors are Congenital Mesoblastic Nephroma which presents at birth or below 6 months of age, has ETV6-NTRK3 gene fusion, Clear Cell Sarcoma (poor prognosis) and BCOR gene, and the Rhabdoid Tumor which has loss of SMARCB1 (1N1 – deficient tumor)