title: Renal medullary carcinoma response to chemotherapy: a referral center experience in Brazil.
abstract: 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.
authors.affiliation: Instituto do Câncer de São Paulo, Faculdade de Medicina da Universidade de São Paulo.
authors.forename: Marina Cavalcanti
authors.initials: MC
authors.lastname: Maroja Silvino
authors.forename: Camila Motta
authors.initials: CM
authors.lastname: Venchiarutti Moniz
authors.forename: Gustavo Henrique
authors.initials: GH
authors.lastname: Munhoz Piotto
authors.forename: Sheila
authors.initials: S
authors.lastname: Siqueira
authors.forename: Ariel
authors.initials: A
authors.lastname: Galapo Kann
authors.forename: Carlos
authors.initials: C
authors.lastname: Dzik
keywords: carcinoma; kidney neoplasms; renal cell; renal medullary carcinoma; sickle cell trait