Rapid development of multiple liver metastases in a child with medullary carcinoma of the kidney and sickle cell trait

The clinical course of an 8-year-old patient with metastatic medullary renal cancer and sickle cell trait is presented. We report the youngest child with sickle cell trait who developed renal medullary carcinoma. This report illustrates the aggressive nature of this rare renal malignancy, which is frequently not considered until in an advanced stage. Within three weeks of restaging following initial surgery and chemotherapy he presented with signs of sepsis and disseminated intravascular coagulation attributed to new hepatic metastases. Despite multi-modality therapy, which included surgical debulking of the primary tumor, local irradiation and 2 cycles of chemotherapy with carboplatin and etoposide, the tumor progressed and directly led to his death. While the increased frequency of renal medullary carcinoma in association with sickle cell trait is not well understood, the confluence of these events offers an opportunity to investigate the genetics and biology of this disease and may lead to breakthroughs that will substantially improve the prognosis, as well as detect the disease earlier.