BACKGROUND: Renal medullary carcinoma (RMC) is an aggressive form of non-clear cell kidney cancer that typically affects young adults and is almost exclusively associated with sickle cell trait.
Typical RMC patients tend to be young black males (2:1 male to female predominance) with sickle cell trait who present with pain and hematuria and are found to have metastatic disease at diagnosis. Prognosis is extremely poor, with a mean survival of less than a year in most cases.
CASE REPORT: We present a case illustrative of many of the classic findings of RMC. A 25-year-old female with a past medical history of sickle cell trait presented to the emergency room with right-sided flank pain. Initially, a computed tomography (CT) scan showed only a benign-appearing cystic lesion 4.5 cm in diameter in the right kidney. Six months later, the patient re-presented with hematuria, lightheadedness, and dizziness. A repeat CT scan showed that the renal cyst had enlarged to 5.6 cm. The patient underwent a decortication and fulguration procedure that revealed malignancy in the wall of the cyst. She was given a series of treatments but experienced progressive disease and passed away 2 years and 8 months after metastatic diagnosis.
CONCLUSION: Neither systemic therapy nor radiation therapy has been found to be particularly efficacious in the treatment of RMC. As the underlying biologic mechanisms of RMC are further elucidated on a basic science level, we expect new translational research opportunities will emerge and novel treatment options will be developed for this rare and dismal disease.
Written by:
Shetty A, Matrana MR. Are you the author?
Department of Genitourinary Medical Oncology, University of Texas MD Anderson Cancer Center, Houston, TX; Department of Hematology and Oncology, Ochsner Clinic Foundation, New Orleans, LA.
Reference: Ochsner J. 2014 Summer;14(2):270-5.
PubMed Abstract
PMID: 24940141
UroToday.com Renal Cancer Section
