[Renal tumors of childhood and adolescence associated with chromosomal anomalies].
The pediatric kidney is a common site for tumors carrying specific chomosomal alterations. The most common of these is the nephroblastoma or Wilms tumor (WT), which is associated with anomalies in two loci: 11p13 and 11p15, the latter also linked to Beckwith-Wiedemann syndrome. Two other genes that seem to be implicated are WT3 and WT4. In addition, 1q gains or 22 deletions have been shown to independently be associated with a worst prognosis in WTs. Other neoplasms with chromosomal rearrangements, such as Renal Cell carcinomas (CRs) are much less frequent in children (between 1.8 and 6.3% of all malignant renal tumors). Among these, the "translocation renal carcinomas" have been identified involving the locus Xp11 with two main types of translocations: t(X;1), and t(X; 17). Congenital mesoblastic nephroma (NMC) is a renal tumor affecting newborns and young infants. NMCs of the cellular type feature a specific translocation t(12; 15)(p13;q25), which is also present in congenital fibrosarcomas outside of the kidney. These findings have led to conclude that these two tumors (NMC and congenital fibrosarcoma) are genetically equivalent. Rhabdoid tumors (TRs) of the kidney are very rare and aggressive neoplasms that appear with a mean age of 11 months. At least 50% of these TRs carry abnormalities in the hSNF5/INI1 gene, at 22q11.2. This gene is probably involved in transcriptional modulation of other genes such as the oncogene c-Myc, and also of the retinoblastoma protein RB signaling pathway.
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Program of Pediatric and Developmental Pathology, Yale University School of Medicine, New Haven 06520-8023, USA.
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D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002869:Chromosome Aberrations; D006801:Humans; D007223:Infant; D007680:Kidney Neoplasms / Q000235:genetics*; D009396:Wilms Tumor / Q000235:genetics