High-dose-intensity MVAC for Advanced Renal Medullary Carcinoma: Report of Three Cases and Literature Review.

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.