No coin nor oath required. For personal study only.
Our objechves include: i) determirung correlations between Wilms' tumor-specific molecular genehc events and clinicopathologc features; (ii) identifyrig hrther DNA/chromosomal aberrations; and (iii) establishing a Wilms' tumor tissue bank. To this end, a Witms' tumor biology protocol has been developed through the Pediatric Oncology Group (PDC). Appro-tely 120 cases have ben acaued to date, with tumor and c o n s t i t ~t i o ~l DNA available for all, and adjacent normal kidney and parental DNA for many. As expected, approximately 40% manifest loss of heterozygosity GOH) for chromosome l l p l j markers, including Ilp13 markers in 29%. We have identified LOH for chromosome I6q m 17/107 (16%) ~11ses. and p r e l i m i ~r y analysrs may indicate LOH for lp in 4/40 (10%) and duplication 1q in 6/40 (15%). The smallest region of overlap between deletions lies between lbq2l and q23. There is no bias to parental o r i p of the 1Mt allele. EventuUy we will correlate LOH for all 5 of these regions in 250 cases with pathologic parameters including presence and type of nephrogmic rests, predominant histology, tumor and disease stage, microsubstaging features, congenital anomalies and relapsefree survival. Correlations will be determined in collaboration with the Nahonal Wilms Tumor Study. Because of the assodation of chromosome 11 miparental disomy NPD) with Beckwith-Wiedemann syndrome, we have assessed whether UPD might be a more common mechanism predisposing to sporadic Wiims' tumor. In an analysis of constitutional DNA fmm 30 W W tumor patients and their parents, UPD for chromosome 11 can be ruled out in all 30 and therefore is not frequent The Poc Wilms' tumor bank is open to any investigator. Justification of scientific merit, establishment of feasibility and ciinical/sdentific relevance are the only criteria used by the FOG W W Tumor Biology Subcommittee in approving requests for biologic samples. kTL. M. 5' TUMOR AND PARENTAL AGE: =?CRY FRCM THE NATIONAL WILMS' TUMOR STUDY Jane !!. Olson", Normar. L. B r e s i o d a n d J. Bruce Beckwifh** *Depar:menc o f B i o s r a t i s : i c s , Univ. o f Nashingron **Depar:men: of Parhology, The C h i l d r e n ' s H o s p i t a l , Denver Age d i s t r i b u t i o n s o f p a r e n t s a t b i r t h of p a t i e n t s r e g i s t e r e d i n :he National Wilms' Tumor Study were compared t o those of t h e general popu1a:ion.
An i n c r e a s i n g incideiice o f s p o r a d i c Idilms' tumor wich i n c r e a s i n g p a t e r n a l age w a s found, w i t h a r e l a r i v e r i s k of 2 . 1 of tumor i n c h i l d r e n o f f a t h e r s over 55
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The Workshop addressed under three rubrics the progress made and future directions in research conceming primary renal tumors of childhood. ## MOLECULAR BIOLOGY AND GENETICS Current information implicates involvement of four distinct loci in the development of Wilms tumor. One locus is at chromos