Effect of Duration of Treatment on Treatment Outcome for Patients With Clear-Cell Sarcoma of the Kidney: A Report From the National Wilms' Tumor Study Group

• Published in: Journal of clinical oncology Vol. 22; no. 3; pp. 468 - 473
• Main Authors: Seibel, Nita L., Li, Sierra, Breslow, Norman E., Beckwith, J. Bruce, Green, Daniel M., Haase, Gerald M., Ritchey, Michael L., Thomas, Patrick R.M., Grundy, Paul E., Finklestein, Jerry Z., Kim, Tae, Shochat, Stephen J., Kelalis, Panayotis P., D'Angio, Giulio J.
• Format: Journal Article
• Language: English
• Published: Baltimore, MD American Society of Clinical Oncology 01.02.2004; Lippincott Williams & Wilkins
• Subjects: Adolescent; Adult; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biological and medical sciences; Child; Child, Preschool; Dactinomycin - administration & dosage; Disease-Free Survival; Doxorubicin - administration & dosage; Female; Humans; Infant; Infant, Newborn; Kidney Neoplasms - drug therapy; Kidney Neoplasms - mortality; Kidney Neoplasms - pathology; Kidneys; Male; Medical sciences; Middle Aged; Neoplasm Recurrence, Local - prevention & control; Neoplasm Staging; Nephrology. Urinary tract diseases; Sarcoma, Clear Cell - drug therapy; Sarcoma, Clear Cell - mortality; Sarcoma, Clear Cell - pathology; Survival Rate; Time Factors; Treatment Outcome; Tumors; Tumors of the urinary system; Vincristine - administration & dosage; Kidney disease; Human; Urinary system disease; Cancerology; Prognosis; Treatment; Urinary system; Wilms tumor; Clear cell sarcoma; Malignant tumor
• ISSN: 0732-183X; 1527-7755
• DOI: 10.1200/JCO.2004.06.058

To evaluate the effect of conventional and standard (ST) versus pulse-intensive (PI) chemotherapy and short-duration versus long-duration chemotherapy on relapse-free survival (RFS) and overall survival rates of patients with clear-cell sarcoma of the kidney (CCSK) entered onto the National Wilms' Tumor Study (NWTS)-4. The 5-year and 8-year RFS rates were determined for patients with CCSK treated on the NWTS-4. After August 6, 1986, 40 previously untreated children younger than 16 years with CCSK were randomly assigned, after the completion of 6 months of chemotherapy, to discontinue (short) or continue 9 additional months (long) of treatment with chemotherapy regimens that included vincristine and either divided-dose (ST) courses (5 days) or single-dose (PI) treatment with dactinomycin and divided-dose (ST) courses (3 days) or single-dose (PI) treatment with doxorubicin. For patients with CCSK, the 5- and 8-year RFS rates were 65.2% and 60.6%, respectively, for patients randomly assigned to the short chemotherapy and 87.8% (both 5- and 8-year RFS) for patients randomly assigned to the long chemotherapy (P =.08). The overall survival rates for patients at 5 and 8 years were 95.5% and 85.9%, respectively, for the short chemotherapy and 87.5% (both 5- and 8-year overall survival) for the long chemotherapy (P =.99). In NWTS-4, the overall survival rates for patients with CCSK improved from NWTS-3 (83% v 66.9% at 8 years, respectively; P <.01). CCSK patients exhibit an improved RFS from a longer course of therapy when using vincristine, doxorubicin, and dactinomycin, but their long-term survival is unchanged compared with patients receiving 6 months of therapy. The overall survival rates for patients with CCSK have improved from NWTS-3.