Management of adults with Wilms tumor: recommendations based on international consensus [Abstract]

Purpose: Wilms tumor is exceedingly rare in adults, with only approximately 11 cases expected annually per 100 million population in Western Europe and North America. Most adult patients will be diagnosed unexpectedly following nephrectomy for presumed renal cell carcinoma. Due to its extreme rarity, no standard therapeutic recommendations are available so far. Population-based analyses show that outcome for adults with Wilms tumor is inferior compared to children, although better results are reported when treated within pediatric trials. While the reasons for this are multifactorial, institutional experiences suggest that unfamiliarity of adult oncologists and pathologists with this childhood tumor delays in initiating chemotherapy and applying appropriate risk-adapted treatment are contributing factors. Method: To improve outcome through standardized treatment an international consensus guideline for the management of adults withWilms tumor is proposed. The guideline builds on discussions held during the SIOP 2003 congress in Cairo, where representatives of the renal tumors committees of SIOP and the Children’s Oncology Group (COG) were present and has been updated with a review of more recently published institutional and trial experience of adults treated on pediatric protocols. Results: The proposed guidelines provide a critical evaluation of the current evidence for clinical decision making in the management ofWilms tumor in adults and propose details of how the current pediatric therapeutic recommendations should be adapted for use in adults. Moreover, we propose a prospective international registry to improve the evidence base. Conclusion: A standardized approach to the diagnosis, staging and treatment of adults with Wilms tumor is proposed based on available literature and international expert consensus from the field of pediatricWilms tumor. The aim is to limit treatment delay after surgery and encourage a uniform approach to the management of this very rare disease and thereby to improve survival