Renal medullary carcinoma and sickle cell trait: A systematic review

Sickle cell trait (SCT) carries a small risk of renal medullary carcinoma (RMC). We conducted a systematic literature review and reported new four RMC cases (total N = 217). Eighty eight percent had SCT and 8% had sickle cell disease; 50% were children. Males had 2.4× risk than females. Isolated hematuria or in combination with abdominal or flank pain was the presenting sign in 66% cases. Tumor‐related mortality was 95%. Four non‐metastatic patients were long‐term disease‐free survivors. Although risk appears to be very low , individuals with SCT should be informed about the low risk of RMC with the hope of early diagnosis. Hematuria should prompt immediate investigation. Pediatr Blood Cancer 2015;62:1694–1699. © 2015 Wiley Periodicals, Inc.