TY - JOUR
T1 - The significance of sarcomatoid and rhabdoid dedifferentiation in renal cell carcinoma
AU - Hahn, Andrew W.
AU - Lebenthal, Justin
AU - Genovese, Giannicola
AU - Sircar, Kanishka
AU - Tannir, Nizar M.
AU - Msaouel, Pavlos
N1 - Publisher Copyright:
© 2022 The Author(s)
PY - 2022/1
Y1 - 2022/1
N2 - 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.
AB - 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.
KW - Dedifferentiation
KW - Immune checkpoint therapy
KW - ISUP grade
KW - Renal cell carcinoma
KW - Rhabdoid
KW - Sarcomatoid
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U2 - 10.1016/j.ctarc.2022.100640
DO - 10.1016/j.ctarc.2022.100640
M3 - Article
C2 - 36174377
AN - SCOPUS:85138812625
SN - 2213-0896
VL - 33
JO - Cancer Treatment and Research Communications
JF - Cancer Treatment and Research Communications
M1 - 100640
ER -