title: Renal medullary carcinoma and sickle cell trait: A systematic review.
abstract: 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.
authors.affiliation: Division of Pediatric Hematology-Oncology, University of Miami, Miami, Florida.
authors.forename: Ofelia
authors.initials: O
authors.lastname: Alvarez
authors.affiliation: Division of Pediatric Pathology, University of Miami, Miami, Florida.
authors.forename: Maria M
authors.initials: MM
authors.lastname: Rodriguez
authors.affiliation: Department of Public Health Sciences, University of Miami, Miami, Florida.
authors.forename: Lanetta
authors.initials: L
authors.lastname: Jordan
authors.affiliation: Division of Pediatric Hematology-Oncology, Children's Hospital of Michigan, Detroit, Michigan.
authors.forename: Sharada
authors.initials: S
authors.lastname: Sarnaik
mesh_terms: D018276:Carcinoma, Medullary / Q000235:genetics*; D020022:Genetic Predisposition to Disease / Q000235:genetics*; D006801:Humans; D007680:Kidney Neoplasms / Q000235:genetics*; D012805:Sickle Cell Trait / Q000235:genetics*
keywords: cancer; rare tumors; renal; sickle cell disease; sickle cell trait; tumors