Cystic presentation of a renal medullary carcinoma in a young woman

Renal medullary carcinoma ( RMC) is a rare tumor, originating in the epithelial papillary cells, that primarily affects young black men with sickle cell trait. We report the case of a 29-year-old Caucasian woman, who at ultrasound showed a cystic mass at the right kidney, with a vascular pattern at the level of the cystic wall. A CT-guided biopsy of the lesion was performed, revealing the presence of unspecified tumor cells. A total nephrectomy was then performed. Microscopically, a tumor resulting from the epithelial papillary cells was observed; several areas of lymphoplasmacytic infiltrates were seen at the junction between the normal kidney tissue and the tumor, and a renal medullary carcinoma was diagnosed. Six months later, the patient developed multiple pulmonary metastases and started chemotherapy with Carboplatin, Paclitaxel and Gemcitabine. The patient died after 27 months of follow-up. RMC is a tumor that usually occurs in young black men, at an average age of 25 years with extremes of 11 and 39 years. Sickle-cell disease or sickle-cell trait is often present. The longest documented survival ( until now) for RMC was 16 months. The best treatment is surgery with enlarged nephrectomy. There are still no appropriate chemotherapy protocols.