Bilateral Wilms’ Tumors

Abstract

AbstractBilateral Wilms’ tumor (BWT), labelled as stage V, is different, is more challenging, and currently has a significantly worse prognosis than unilateral Wilms’ tumor (uWT). On imaging, tumor on one side associated with a single lesion of more than 1 cm or multiple lesions of any size in the contralateral kidney is considered BWT. It may be synchronous about 3.6 to 8%, or metachronous (0.85%). It may be prudent to treat patients with contralateral nephrogenic rests or children with predisposing syndromes or germline mutations on the lines of BWT to maximize salvage of renal function in the long run. The aims of management of BWT are to improve survivals, while maximizing renal tissue preservation and thereby renal function using appropriate multimodal therapy. The currently recommended surgery is bilateral nephron sparing surgery (NSS) to maximize renal preservation and function. Neoadjuvant chemotherapy (ChT) may be based on imaging studies (SIOP and COG). SIOP recommends neoadjuvant Vincristine and Actinomycin-D for 4 weeks. However, if disease is stable or progressive, escalation with the addition of Doxorubicin, for a total duration of up to 12 weeks followed by surgery is advised. COG (AREN0534) and ICMR guidelines currently recommend administering three drugs for a maximum of 12 weeks. Postoperative management includes adjuvant ChT based on histological risk stratification and staging. XRT is indicated for patients with positive tumor margins, positive lymph nodes, and metastatic disease sites.At the end of study of this chapter, the reader is expected to have a clear understanding of the current scenario of BWT, suspect and anticipate BWT based on predisposing factors and risks. The reader should be able to provide appropriate investigations, take rapid decisions on treatment plans, and know the intricacies of preoperative strategies, intraoperative techniques, and post-operative management. This should lead to improved renal salvage and hence renal function with better event-free survival and overall survival (OS). The need of the hour is to improve renal preservation with appropriate preoperative ChT and prevent prolonged ChT in the hope of shrinking the tumor. The future and advances in various fields including MRI diffusion studies and molecular studies vis-a-vis decision-making will also be touched upon.KeywordsBilateral Wilms’ tumorSIOP: AREN 0534Nephron sparing surgeryNephroblastomatosisOncological outcomesRenal functional outcomes