Renal medullary carcinoma in a six-year-old boy with sickle cell trait

Pathology & Oncology Research

Araceli Solis Coria¹  Cirilo Sotelo-Avila²  Roberto Vargas-Gonzalez³ 

[1] Hospital Infantil de México Federico Gomez$$;Cardinal Glennon Children’s Hospital$$;Hospital Para el Niño Poblano$$

关键词: collecting duct carcinoma;   

DOI  :  10.1007/BF03033737

学科分类：生理学与病理学

来源: Springer

【 摘 要 】

Renal medullary carcinoma (RMC), an aggressive malignant epithelial neoplasm, first emerged as a distinct clinicopathologic entity in 1995. It affects individuals 40 years of age or younger and is strongly associated with sickle cell disease or trait. The majority of patients with RMC have widely disseminated disease at the time of diagnosis and most fail to respond to both chemotherapy and radiotherapy. Mortality approaches 100%, and death usually occurs within a few months to a year of diagnosis. We report a 6-year-old African-American boy with a history of gross hematuria who died four weeks after diagnosis of disseminated metastatic disease. Autopsy showed a 4.4-cm renal mass with metastases to the contra lateral kidney, liver, lungs and multiregional lymph nodes. RMC should be included in the differential diagnosis of any patient 40 years old or younger with a history of hemoglo-binopathy and gross hematuria and/or abdominal or flank pain. A brief discussion of the differential diagnosis, histogenesis and treatment is presented in this study.

【 授权许可】

Unknown