CDKN2A

Targeted therapy

Biology

Angiogenesis

Clear cell

Cancer research

Renal cell carcinoma

Immunology

Genetics

Internal medicine

Chemotherapy

Oncology

Medicine

Immunotherapy

Nivolumab

Immunohistochemistry

Cancer

Pathology

Sarcomatoid carcinoma

Cancer Treatment and Research Communications

Dedifferentiation in renal cell carcinoma (RCC), either sarcomatoid or rhabdoid, is an infrequent event that may occur heterogeneously in the setting of any RCC histology and is associated with poor outcomes. Sarcomatoid dedifferentiation is associated with inferior survival with angiogenesis targeted therapy and infrequent responses to cytotoxic chemotherapy. However, immune checkpoint therapy has significantly improved outcomes for patients with sarcomatoid dedifferentiation. Biologically, sarcomatoid dedifferentiation has increased programmed death-ligand 1 (PD-L1) expression and an inflamed tumor microenvironment, in addition to other distinct molecular alterations. Less is known about rhabdoid dedifferentiation from either a clinical, biological, or therapeutic perspective. In this focused review, we will discuss the prognostic implications, outcomes with systemic therapy, and underlying biology in RCC with either sarcomatoid or rhabdoid dedifferentiation present.

The significance of sarcomatoid and rhabdoid dedifferentiation in renal cell carcinoma