Syndromic Wilms tumor: a review of predisposing conditions, surveillance and treatment

Translational Andrology and Urology
Esther K Liu, Kristina D Suson

Abstract

Predisposing syndromes associated with an increased risk of Wilms tumor (WT) are responsible for 9-17% of all cases of the malignancy. Due to an earlier age at WT diagnosis and an increased incidence of bilateral and metachronous disease, management of syndromic WT warrants a distinct approach from that of non-syndromic WT. This review of English-language manuscripts about WT focuses on the most common syndromes, surveillance protocols and current treatment strategies. Highlighted syndromes include those associated with WT1, such as WAGR (Wilms-Aniridia-Genitourinary-mental Retardation), Denys-Drash syndrome (DDS), and Frasier syndrome, 11p15 defects, such as Beckwith-Wiedemann syndrome (BWS), among others. General surveillance guidelines include screening renal or abdominal ultrasound every 3-4 months until the age of 5 or 7, depending on the syndrome. Further, some of the predisposing conditions also increase the risk of other malignancies, such as gonadoblastoma and hepatoblastoma. With promising results for nephron-sparing surgery in bilateral non-syndromic WT, there are increasing reports and recommendations to pursue nephron-sparing for these patients who are at greater risk of bilateral, metachronous lesions. In addition...Continue Reading

Citations

May 7, 2021·Acta Biochimica Et Biophysica Sinica·Qiang YuZhengtuan Guo
Jun 19, 2021·Der Radiologe·J-P SchenkN Graf
Aug 21, 2021·Seminars in Pediatric Surgery·Fuchs JWarmann Sw
Aug 21, 2021·Journal of the National Comprehensive Cancer Network : JNCCN·Frank BalisMiranda Hughes

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