Renal medullary carcinoma and sickle cell trait: A systematic review

Pediatr Blood Cancer. 2015 Oct;62(10):1694-9. doi: 10.1002/pbc.25592. Epub 2015 Jun 5.


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.

Keywords: cancer; rare tumors; renal; sickle cell disease; sickle cell trait; tumors.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Carcinoma, Medullary / genetics*
  • Genetic Predisposition to Disease / genetics*
  • Humans
  • Kidney Neoplasms / genetics*
  • Sickle Cell Trait / genetics*