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OBJECTIVES - Renal medullary carcinoma is a rare and highly aggressive cancer that is characteristically resistant to most forms of chemotherapy. This pediatric tumor, exclusively affecting individuals who carry at least one sickle hemoglobin allele, carries a uniformly fatal outcome, usually within a matter of weeks, and no effective therapies have been reported.
METHODS - We report on the use of a high-dose-intensity regimen of methotrexate, vinblastine, doxorubicin, and cisplatin in 3 patients.
RESULTS - All 3 patients were able to tolerate multiple cycles of therapy and achieved at least a partial response and longer survival than predicted from the historical precedent.
CONCLUSIONS - Our results have shown that this regimen provides a tolerable therapy with efficacy to promote survival for several months and suggest that highly intensive regimens of chemotherapy might bring additional benefit to the young patients with these rare tumors.