title: Cystic presentation of a renal medullary carcinoma in a young woman.
abstract: 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.
authors.affiliation: Department of General Surgery and Organ Transplantation, Sapienza University of Rome, Rome - Italy.
authors.forename: Giovanni Battista
authors.initials: GB
authors.lastname: Levi Sandri
authors.forename: Gabriele
authors.initials: G
authors.lastname: Spoletini
authors.forename: Quirino
authors.initials: Q
authors.lastname: Lai
authors.forename: Gianluca
authors.initials: G
authors.lastname: Mennini
authors.forename: Massimo
authors.initials: M
authors.lastname: Rossi
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D016190:Carboplatin / Q000008:administration & dosage; D018276:Carcinoma, Medullary / Q000150:complications / Q000000981:diagnostic imaging / Q000556:secondary / Q000628:therapy*; D003841:Deoxycytidine / Q000008:administration & dosage / Q000031:analogs & derivatives; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D052177:Kidney Diseases, Cystic / Q000000981:diagnostic imaging / Q000209:etiology* / Q000628:therapy*; D007680:Kidney Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology / Q000628:therapy*; D008175:Lung Neoplasms / Q000000981:diagnostic imaging / Q000556:secondary / Q000628:therapy*; D009392:Nephrectomy / Q000379:methods; D017239:Paclitaxel / Q000008:administration & dosage; D012307:Risk Factors; D000093542:Gemcitabine
chemical_list: D003841:Deoxycytidine; D016190:Carboplatin; D017239:Paclitaxel; D000093542:Gemcitabine