Renal
medullary carcinoma (RMC) is rare, accounting for less than 1% of all
renal neoplasms. Case reports suggest RMC is highly aggressive, poorly responsive to
chemotherapy, often metastatic at diagnosis, affects young men with
sickle cell trait, and median overall survival (mOS) is less than 12 months. We report the epidemiological characteristics, treatments performed, response rate to each treatment and mOS of five patients with RMC. All patients had
sickle cell trait, four were male, three had metastatic disease at diagnosis and mean age at diagnosis was 25 years. Non-metastatic patients were submitted to
nephrectomy. Two patients had partial response to first line
chemotherapy including
cisplatin and
gemcitabine. There was no response to
sunitinib or second line chemo -
therapy; mOS was 6 months. Due to its rarity, case series are the only evidence available to discuss the treatment for RMC. In our experience, only
cisplatin and
gemcitabine based regimen offered response.