title: Renal Medullary Carcinoma and Sickle Cell Trait: A Push for Early Diagnosis and Intervention Report of Two Cases.
abstract: Renal medullary carcinoma (RMC) is a rare but highly aggressive neoplasm that primarily affects young African Americans with sickle cell trait. Most patients present with macroscopic hematuria and have metastases at diagnosis. Chemotherapy, biologics directed against the more common renal cell carcinomas and radiation have all shown limited efficacy in treating patients with advanced RMC. We report two patients with RMC. Both had Stage IV disease. One underwent radical nephrectomy followed by radiation and biologic drug therapy but died five months later; the other underwent multiple cycles of chemotherapy plus anti-angiogenesis treatment but died 15 months after diagnosis. Review of the literature suggests that early diagnosis and surgical intervention while the tumor is confined to the kidney offer the best prospect for long term survival. Since newborn screening for sickle cell is now mandated in the US, the at-risk population for RMC could be identified and followed by yearly urine dipstick testing for microscopic hematuria. Those who test positive can be further evaluated to rule out RMC.
authors.affiliation: Department of Pathology, Mercer University School of Medicine, Macon, GA, United States.
authors.forename: Spencer Alexander
authors.initials: SA
authors.lastname: Carter
authors.affiliation: Department of Pathology, Mercer University School of Medicine, Macon, GA, United States. Electronic address: walker_an@mercer.edu.
authors.forename: Anna N
authors.initials: AN
authors.lastname: Walker
mesh_terms: D000328:Adult; D020533:Angiogenesis Inhibitors / Q000008:administration & dosage*; D000970:Antineoplastic Agents / Q000008:administration & dosage*; D018276:Carcinoma, Medullary / Q000150:complications / Q000473:pathology / Q000628:therapy; D002292:Carcinoma, Renal Cell / Q000473:pathology / Q000628:therapy; D055088:Early Detection of Cancer; D017809:Fatal Outcome; D005260:Female; D006417:Hematuria / Q000175:diagnosis*; D006801:Humans; D007680:Kidney Neoplasms / Q000473:pathology / Q000628:therapy; D008297:Male; D009367:Neoplasm Staging; D009392:Nephrectomy / Q000379:methods*; D011379:Prognosis; D011878:Radiotherapy / Q000379:methods*; D012805:Sickle Cell Trait / Q000150:complications / Q000175:diagnosis / Q000652:urine
chemical_list: D020533:Angiogenesis Inhibitors; D000970:Antineoplastic Agents
keywords: Hematuria; Renal medullary carcinoma; Sickle cell disease; Sickle cell trait