The Not-So-Benign Sickle Cell Trait: A Case of Renal Medullary Carcinoma

Sickle cell trait (SCT) has long been considered a benign carrier state with malarial protection, but carriers can be affected by increased venous thromboembolism, exercise-related injury, renal complications and very rarely a fatal renal malignancy. Renal medullary carcinoma is a very rare and aggressive renal tumor described almost exclusively in sickle cell trait. A review of the current literature provides clues to this link and describes trends expected in these cases. We report a case of renal medullary carcinoma in a 32-year-old female with known sickle trait who presented with cough, hemoptysis, left flank pain and gross hematuria. Initial presentation was concerning for pulmonary renal syndrome, but her labs did not show evidence of nephritic syndrome with negative autoimmune and infectious serologies. Abdominal CT imaging identified a large left renal mass with biopsy confirmation of renal medullary carcinoma and subsequent staging showing pulmonary and osseous metastases. Despite palliative chemotherapy, she died within 3 months of diagnosis following a protracted clinical course. Renal medullary carcinoma should be considered in patients with SCT presenting with hematuria.