Purpose: Renal medullary carcinoma is a rare and extremely aggressive neoplasm that almost always develops in young patients with sickle cell trait. To our knowledge all cases to date have been metastatic at surgical resection. Pathological examination reveals an aggressive tumor mainly involving the renal medulla with a varied morphology. The prognosis is dismal. Mean survival from the time of resection is 15 weeks (range 2 to 52). The disease course has not been altered by surgery, radiotherapy or various regimens of chemotherapeutic agents. Materials and Methods: We add to the literature our experience treating renal medullary carcinoma in 2 cases and review the existing literature on this disease. Results: Both patients whom we treated died of the disease, as have the other 35 patients described in the literature. Conclusions: A high index of suspicion may lead to earlier diagnosis and treatment, and survival of patients with renal medullary carcinoma.
- Sickle cell trait