CA: A Cancer Journal for Clinicians, Vol 24, 66-77, Copyright
© 1974 by American Cancer Society
Wilms' Tumor—A Clinical Review
V. C. Canale M.D.1 and
E. C. Muecke M.D.2
1 Associate Professor of Pediatrics, Department of Pediatrics, Division of Pediatrics Hematology, New York Hospital—Cornell Medical Center, New York, New York.
2 Attending Surgeon (Urology) and Professor of Surgery (Urology), New York Hospital—Cornell Medical Center.
The National Wilms' Tumor Study, organized in 1969, is now evaluating whether routine postoperative radiation therapy is necessary for patients with well encapsulated, localized, completely resected lesions. It is also evaluating which of the two effetive chemotherapeutic agents for Wilms' tumor gives the best result and whether survival can be improved by their combined use. (Table 5.) Data on 257 patients with Wilms' tumor have revealed that: (1) there are approximately one and a half times as many children in Groups I and II as in Groups III and IV; (2) the incidence of bilateral tumor is low (about 5 percent); and (3) there has not been an unduly high rate of recurrence in the nonirradiated children.
Using the described methods of management, the mortality rate for children with Stages II to IV Wilms' tumor still ranges between 20 to 50 percent. The survival rate is related closely to the patient's age, extent of disease at the time of diagnosis, and the presence of pulmonary metastases.
In patients under one year of age, the two-year survival is 85 percent and can be equated with a cure. Survival statistics for children over one year of age have improved with the increasing number of modalities of therapy available. iscovemycin,ry of rc 25 percent of patients survived the two-year risk period; with actinomycin, 58 to 84.6 percent of child successfully survive this period, if no metastases are present at operation. Long-term survival is only 12.8 percent if metastases are evident. More vigorous chemotherapy and surgical resection of metastases in the future may contribute to better prognosis.
Normal renal function in the unaffected kidney of 108 children who underwent nephrectomy, postoperative irradiation and chemotherapy has been reported. However, if more than 1200 rads of radiation are given, some form of irradiation nephritis is inevitable. Although the incidence of urinary tract infections in the remaining kidney is higher than in normal children, no impairment of renal function has ensued, [see table-6 in source pdf] if adequate and prompt treatment is administered.
Complications of intensive therapy for Wilms' tumor include severe portal hypertension, radiation nephritis, and kyphoscoliosis. In view of the modes of spread of the tumor and the potential complications of radiotherapy and chemotherapy, follow-up biochemical, urinary, and radiographic observations are mandatory. (Table 6.)
