General Discussion: 40 year old male with no known medical history was found to have enlarged retrocrural lymph nodes which were subsequently biopsied and found to be poorly differentiated carcinoma. Based on the location and appearance of the lesion, the patient was tested for sickle trait and found to be positive. Review of the original lymph node pathology led to a presumptive diagnosis of medullary carcinoma of the kidney.
In 1995, Davis et al (1) first described renal medullary carcinoma as the seventh nephropathy of sickle cell disease. The cancer is usually large and metatstatic by the time of detection. It often presents with flank pain and hematuria. The disease has a strong association with sickle cell trait but not with sickle cell disease. Various cytogenetic abnormalities have been found in renal medullary carcinoma, including abnormalities of chromosome 11p, where the beta-globin gene is located. However, no definitive direct chromosomal or environmental link has been established as the cause of this disease. Like in this patient, several studies have shown renal medullary carcinoma has a striking tendancy to be right sided. The most common sites of metastasis are the lymph nodes, lungs, liver, and peritoneum. Many treatments have been tried including surgery, various chemotherapies, and radiation. However, the prognosis remains dismal with medial survival four months from diagnosis (2).
