Maroja Silvino Marina Cavalcanti, Venchiarutti Moniz Camila Motta, Munhoz Piotto Gustavo Henrique, Siqueira Sheila, Galapo Kann Ariel, Dzik Carlos
Instituto do Câncer de São Paulo, Faculdade de Medicina da Universidade de São Paulo.
Rare Tumors. 2013 Aug 20;5(3):e44. doi: 10.4081/rt.2013.e44. eCollection 2013.
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.
肾髓质癌(RMC)很罕见,占所有肾肿瘤的比例不到1%。病例报告表明,RMC具有高度侵袭性,对化疗反应不佳,在诊断时往往已发生转移,影响具有镰状细胞性状的年轻男性,中位总生存期(mOS)不到12个月。我们报告了5例RMC患者的流行病学特征、所接受的治疗、每种治疗的反应率和mOS。所有患者均具有镰状细胞性状,4例为男性,3例在诊断时已有转移性疾病,诊断时的平均年龄为25岁。非转移性患者接受了肾切除术。2例患者对包括顺铂和吉西他滨在内的一线化疗有部分反应。对舒尼替尼或二线化疗无反应;mOS为6个月。由于其罕见性,病例系列是可用于讨论RMC治疗的唯一证据。根据我们的经验,只有基于顺铂和吉西他滨的方案有反应。