The treatment of Wilms' tumor. Results of the national Wilms' tumor study

Abstract

The National Wilms' Tumor Study, initiated in 1969, tested competing treatment strategems for patients with tumors ranging from Group (Gp) I (tumors confined to the kidney and totally removed) to Gp IV (remote metastases present at diagnosis). Three hundred and fifty‐nine of 606 registered patients were randomized in the trial. Gp I patients under 2 years of age fared well whether postoperative radiation therapy (RT) was or was not added to 15 months' maintenance actinomycin D (AMD). Their prognosis was better than that for older cohorts similarly treated, in whom the difference in relapse rates between treatment groups were suggestive of an RT effect. Combined AMD and vincristine (VCR) gave better results than either agent alone in patients with more advanced tumors (Gps II and III) still confined to the abdomen, all of whom received postoperative RT as well. Preoperative VCR given Gp IV patients in addition to postoperative RT, AMD, and VCR did not improve results. The frequency of mesoblastic nephroma (1%), of bilateral tumors (5%), and of incorrect preoperative diagnosis of Wilms' tumor (5%), the toxicities of the various regimens, and other ancillary data are presented and discussed.

A study of surgical profile of patients with Wilms' tumor

“…8 D'Angio et al noted equal incidence on both sides and bilateral tumours in 5%. 7 We found equal incidence on both side similar to that of D' Angio et al finding 7 …”

“…5 Howard et al reported 75% incidence between the age of 1 and 5 years, and 90% below the age of 7 years. 1 We had 92% of cases below the age of 7 years which similar to that of D'Angio, 7 Harward et al 1 Incidence for Wilms' tumor was almost equal (0.97:1) in series of Bennington. 4 Bernard 5 reported M:F ratio of 1:1.7.…”

“…7 In Bernards series maximum cases were below the age of 6 years (85%). 5 Howard et al reported 75% incidence between the age of 1 and 5 years, and 90% below the age of 7 years.…”

Familial Bilateral Wilms’ Tumor

“…There is a variable degree of penetration in cases involving familial Wilms' tumor. This phenomenon has been previously reported in twins [5,9], but has been reported in only one of two monozygote twins and in a younger brother [6]. There is a much higher incidence rate of bilateral renal tumors in familial cases.…”

“…The conservative resection protocol adapted for cases of bilateral Wilms' tumor according to NWTS III should be followed, but with special reservation which has been reported by Laberge et al [7]. It is well known that results of Wilms' tumor treatment are among the best in infant neoplasms [9]; the higher cure rate is due mainly to improved surgical procedures, as well as the sensitivity of WT to chemotherapeutic agents and radiotherapy. BWT usually has a favorable histology in younger patients.…”