Recent Advances in Renal Medullary Carcinoma

Renal medullary carcinoma (RMC) is a rare renal malignancy that has been associated with sickle hemoglobinopathies. RMC is aggressive, difficult to treat, and occurs primarily in adolescents and young adults of African ancestry. This cancer is driven by the loss of SMARCB1, a tumor suppressor seen in a number of primarily rare childhood cancers (e.g., rhabdoid tumor of the kidney and atypical teratoid rhabdoid tumor). Treatment options remain limited due in part to the limited knowledge of RMC biology. However, significant advances have been made in unraveling the biology of RMC, from genomics to therapeutic targets, over the past 5 years. In this review, we will present these advances and discuss what new questions exist in the field.

Automated summary

Renal medullary carcinoma (RMC) is a rare renal malignancy that primarily occurs in adolescents and young adults of African ancestry. This aggressive cancer is driven by the loss of the tumor suppressor SMARCB1, and limited knowledge of RMC biology has hindered treatment options. However, significant advances have been made in understanding the genomics and therapeutic targets of RMC in the past 5 years.