Renal medullary carcinoma: a case report and brief review of the literature.

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.