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.
journal_name
J Natl Med Assocjournal_title
Journal of the National Medical Associationauthors
Carter SA,Walker ANdoi
10.1016/j.jnma.2016.08.006subject
Has Abstractpub_date
2017-04-01 00:00:00pages
63-65issue
1eissn
0027-9684issn
1943-4693pii
S0027-9684(16)30008-6journal_volume
109pub_type
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