Factors affecting the risk of contralateral Wilms tumor development

Abstract

BACKGROUND Approximately 1% of children with unilateral Wilms tumor develop contralateral disease. The authors assessed the demographic and histologic features associated with metachronous bilateral Wilms tumor (BWT). METHODS Characteristics of all children registered on the first four National Wilms Tumor Studies (NWTS) were recorded. The primary endpoint for evaluation was the first appearance of Wilms tumor in the remaining kidney. The cumulative risk of contralateral disease as a function of time since initial presentation was calculated as 1 minus the Kaplan–Meier estimate of remaining free of contralateral disease. A matched case control study was conducted to determine whether the presence and type of nephrogenic rests (NRs) were associated with metachronous BWT. RESULTS Fifty‐eight of 4669 registered children developed metachronous BWT; 38 of 2445 females (expected, 30.2) versus 20 of 2224 males (expected, 27.8) (P = 0.04) developed BWT. The cumulative incidence of contralateral disease 6 years after initial diagnosis decreased from greater than 3% in the first NWTS to approximately 1.5% in the three subsequent studies (P = 0.08). Patients with NRs had a significantly increased risk of metachronous BWT. This was particularly true for young children (20 of 206 age < 12 months compared with 0 of 304 age > 12 months). Data from the matched case control study confirmed the increased relative risk associated with young age and the presence of NRs. CONCLUSIONS Children younger than 12 months diagnosed with Wilms tumor who also have NRs, in particular perilobar NRs, have a markedly increased risk of developing contralateral disease and require frequent and regular surveillance for several years. Surveillance is also recommended for those with NRs who are diagnosed after the age of 12 months. Cancer 1999;85:1616–25. © 1999 American Cancer Society.

Current Therapy for Wilms’ Tumor

“…Children in whom a contralateral tumor developed more than 18 months after the initial diagnosis had a better OS rate than did those in whom it developed less than 18 months after diagnosis (10-year OS rate, 55.2% versus 39.6%). Children younger than 12 months who have perilobar nephrogenic rests are at markedly increased risk of contralateral disease and require frequent and regular surveillance for several years [65].…”

Beckwith–Wiedemann syndrome

“…3,[10][11][12]13,[15][16][17] Clinical findings associated with higher risks of tumor development include hemihyperplasia, nephromegaly, and nephrogenic rests. 10,18 Although different molecular subgroups have been shown to be associated with different tumor rates and tumor profiles, 19,[20][21][22][23][24] further clinical studies are needed to validate these data before implementing stratified surveillance protocols.…”

Management of Bilateral Wilms Tumours

“…These are preoperative tumour rupture, abdominal lymph node metastases, tumour in the renal vein, multifocal tumour and infiltration into the renal pelvis (10). Nephrogenic rests may be seen in up to 90% of synchronous BWTs and 94% of metachronous BWTs (78% in our series) (11); about 70% of children with synchronous BWT in the NWTS series had multiple nephrogenic rests or nephroblastomatosis (12)(13)(14).…”

“…With synchronous BWT, the traditional approach has been bilateral renal biopsies and staging of each kidney, followed by neoadjuvant chemotherapy and then renal salvage procedures (partial nephrectomy or tumourectomy) (14,22). However, data from NWTS-4 and NWTS-5 have shown conclusively that a significant number of patients have unfavourable histology, which is revealed at the time of definitive surgery following chemotherapy but missed during initial biopsy.…”

“…In BWT, radiotherapy use has decreased over the years; 57% of patients on NWTS-2 and NWTS-3 (12) received renal or renal-bed irradiation, while 42 (21.4%) of the 196 renal units registered on the renal salvage procedure arm of NWTS-4 were treated with radiotherapy (14). Radiation therapy is considered for abdominal stage III tumours or for stage II cases of anaplasia with involved margins at tumour resection.…”