摘要
RMC （renal medullary carcinoma） is considered a rare entity, corresponding to approximately 2% of primary kidney tumors, usually occurring in the second decade of the patient＇s life and it is closely related with previous history of sickle cell disease. Studies have reported RMC as highly aggressive, poorly responsive to chemotherapy, surgery, radiotherapy or targeted therapy. This disease＇s median overall survival is less than 12 months. We are reporting a case of a 21-year-old woman with sickle cell trait, whose initial clinical presentation was RMC already metastatic to the liver. She underwent total right nephrectomy, and her clinical picture worsened during hospitalization with pneumonia, bowel obstruction, oliguria and death in less than two months after diagnosis, with no performance status to attempt any treatment options to her cancer at any time. Although some cases in the literature had reported good response to neoadjuvant therapy, this disease has early recurrence and little response to treatment. Our objective is to call the attention for this aggressive desease to estimulate new perspectives and further studies with chemotherapy and other different kinds of treatment, mainly because only surgical intervention is an incomplete aproach.
