PhD Marry M. van den Heuvel-Eibrink MD,
Paul Grundy MD,
Norbert Graf MD,
Kathy Pritchard-Jones BM, BCh, PhD, FRCPCH,
Christophe Bergeron MD, Catherine Patte MD,
Harm van Tinteren PhD,
Annie Rey Msc,
Carolyn Langford Msc,
Jim R. Anderson Msc, [......],
Paul Grundy,
Norbert Graf,
Kathy Pritchard‐Jones,
Christophe Bergeron,
Catherine Patte,
Harm van Tinteren,
Annie Rey,
Carolyn Langford,
Jim R. Anderson,
Jan de Kraker
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ABSTRACT: Background
To review the clinical characteristics and survival of infants diagnosed with a primary renal tumor in the first 7 months of life.ProcedureA retrospective data review of patients registered in five large international protocols (SFOP/GPOH/SIOP9/93-01, UKW3 and NWTSG 4 and 5) spanning 1985–2002.Results750 (7.2%) of 10,430 registered patients were diagnosed with a renal tumor before age 213 days. Tumor types were Wilms tumor (WT) 58%; congenital mesoblastic nephroma (CMN) 18%; malignant rhabdoid tumor (MRTK) 8%; clear cell sarcoma (CCSK) 2%; non-Wilms tumor (unspecified) 6%; histology unknown, 9%. CMN predominated among tumors diagnosed in the first month of life (54%) but its relative contribution diminished to <10% of all cases diagnosed after the age of 3 months (P < 0.001). Among 639 cases with specified histology and stage, 9/11 stage IV tumors were MRTK, 37/39 bilateral tumors were WT. In 626 children where surgical approach was specified, 522 had immediate nephrectomy. For all cases, 5 years event-free survival (EFS) was 80% and overall survival (OS) 86%. Five years EFS and OS respectively by tumor type were WT (86%, 93%), CMN (94%, 96%), CCSK (49%, 51%), MRTK (16%, 16%).Conclusion
Renal tumors diagnosed in the first 7 months of life generally have an excellent prognosis though histology is an important prognostic factor. In the first 2 months of life the prevalence of CMN is high. The relative occurrence of WT increases rapidly with age thereafter. Bilateral tumors are usually WT. Tumors with metastases at diagnosis are usually MRTK. Pediatr Blood Cancer 2008;50:1130–1134. © 2007 Wiley-Liss, Inc.
Pediatric Blood & Cancer 05/2008; 50(6):1130 - 1134. · 1.89 Impact Factor
