Treatment of stage I Wilms tumor

The AREN0532 (NCT00352534) trial was designed to confirm the findings from NWTS-5 that adjuvant chemotherapy could be omitted for children younger than 2 years at diagnosis with stage I FH Wilms tumor that weighed less than 550 g. A total of 116 patients met the criteria for very low-risk Wilms tumor and were enrolled on the study.[184,185,275]

The AREN0532 (NCT00352534) trial was designed to confirm the findings from NWTS-5 that adjuvant chemotherapy could be omitted for children younger than 2 years at diagnosis with stage I FH Wilms tumor that weighed less than 550 g. A total of 116 patients met the criteria for very low-risk Wilms tumor and were enrolled on the study.[184,185,275]

Twelve patients relapsed.

The AREN0532 (NCT00352534) trial was designed to confirm the findings from NWTS-5 that adjuvant chemotherapy could be omitted for children younger than 2 years at diagnosis with stage I FH Wilms tumor that weighed less than 550 g. A total of 116 patients met the criteria for very low-risk Wilms tumor and were enrolled on the study.[184,185,275]

The estimated 4-year EFS rate was 89.7%, and the OS rate was 100%.

The AREN0532 (NCT00352534) trial was designed to confirm the findings from NWTS-5 that adjuvant chemotherapy could be omitted for children younger than 2 years at diagnosis with stage I FH Wilms tumor that weighed less than 550 g. A total of 116 patients met the criteria for very low-risk Wilms tumor and were enrolled on the study.[184,185,275]

11p15 methylation status was associated with relapse (20% relapse with loss of heterozygosity, 25% relapse with loss of imprinting, and 3.3% relapse with retention of the normal imprinting [P = .011]).

The AREN0532 (NCT00352534) trial was designed to confirm the findings from NWTS-5 that adjuvant chemotherapy could be omitted for children younger than 2 years at diagnosis with stage I FH Wilms tumor that weighed less than 550 g. A total of 116 patients met the criteria for very low-risk Wilms tumor and were enrolled on the study.[184,185,275]

The risk of developing metachronous Wilms tumor is very low in patients with very low-risk Wilms tumor who lack evidence of an underlying syndrome.

The COG reported the outcomes for patients of all ages with stage I FH Wilms tumors showing epithelial-predominant histology. Approximately 20% of stage I FH Wilms tumors registered on AREN03B2 were epithelial predominant. In this group of 177 patients with stage I epithelial-predominant FH Wilms tumors, 117 patients were treated with EE-4A, and 57 patients were classified as having a very low-risk Wilms tumor and were treated with observation only.[201][Level of evidence C1]

The COG reported the outcomes for patients of all ages with stage I FH Wilms tumors showing epithelial-predominant histology. Approximately 20% of stage I FH Wilms tumors registered on AREN03B2 were epithelial predominant. In this group of 177 patients with stage I epithelial-predominant FH Wilms tumors, 117 patients were treated with EE-4A, and 57 patients were classified as having a very low-risk Wilms tumor and were treated with observation only.[201][Level of evidence C1]

The 4-year EFS rate was 96.2%, and the OS rate was 100%.

The COG reported the outcomes for patients of all ages with stage I FH Wilms tumors showing epithelial-predominant histology. Approximately 20% of stage I FH Wilms tumors registered on AREN03B2 were epithelial predominant. In this group of 177 patients with stage I epithelial-predominant FH Wilms tumors, 117 patients were treated with EE-4A, and 57 patients were classified as having a very low-risk Wilms tumor and were treated with observation only.[201][Level of evidence C1]

There was no statistical difference in EFS and OS based on age at diagnosis (<48 months and >48 months) or treatment (EE-4A vs. observation only).

The COG reported the outcomes for patients of all ages with stage I FH Wilms tumors showing epithelial-predominant histology. Approximately 20% of stage I FH Wilms tumors registered on AREN03B2 were epithelial predominant. In this group of 177 patients with stage I epithelial-predominant FH Wilms tumors, 117 patients were treated with EE-4A, and 57 patients were classified as having a very low-risk Wilms tumor and were treated with observation only.[201][Level of evidence C1]

There were six events: three patients developed contralateral tumors after their initial diagnosis, and two of these patients had received adjuvant chemotherapy for their initial tumors. Three patients developed metastatic disease, and all of these patients had previously received EE-4A as their primary therapy.

The AREN0321 (NCT00335556) study demonstrated that outcomes for patients with stage I anaplastic Wilms tumor were improved with the addition of doxorubicin and flank radiation therapy to vincristine/dactinomycin therapy.[274]

The AREN0321 (NCT00335556) study demonstrated that outcomes for patients with stage I anaplastic Wilms tumor were improved with the addition of doxorubicin and flank radiation therapy to vincristine/dactinomycin therapy.[274]

The 4-year EFS and OS rate estimates were 100% in AREN0321, compared with 70% and 81.5%, respectively, in an updated analysis of 27 patients from NWTS-5 (median follow-up, 13.3 years). One patient with diffuse anaplasia relapsed 4.12 years after diagnosis on the AREN0321 trial.

The AREN0321 (NCT00335556) study demonstrated that outcomes for patients with stage I anaplastic Wilms tumor were improved with the addition of doxorubicin and flank radiation therapy to vincristine/dactinomycin therapy.[274]

The addition of doxorubicin and radiation therapy to AREN0321 was based on the pattern of relapse observed in stage I anaplastic Wilms tumor in the abdomen and distant sites in the NWTS-5 trial.

The AREN0321 (NCT00335556) study demonstrated that outcomes for patients with stage I anaplastic Wilms tumor were improved with the addition of doxorubicin and flank radiation therapy to vincristine/dactinomycin therapy.[274]

Retrospective analysis of all patients with stage I anaplastic Wilms tumor treated on NWTS-1 through NWTS-5 and AREN0321 showed a significant improvement in EFS for patients treated with doxorubicin (4-year EFS rate, 97.2% vs. 77.5%; P = .01), but no difference in EFS according to flank radiation therapy was shown (4-year EFS rate, 91.7% vs. 80.2%; P = .15).

The AREN0321 (NCT00335556) study demonstrated that outcomes for patients with stage I anaplastic Wilms tumor were improved with the addition of doxorubicin and flank radiation therapy to vincristine/dactinomycin therapy.[274]

The rate of local recurrence was low (3.6%) and appeared to be similar for patients who received flank radiation therapy (4%) and patients who did not receive flank radiation therapy (6.2%). Local relapse occurred only in patients with diffuse anaplasia.