Next Article in Journal
Negative Impact of Wound Complications on Oncologic Outcome of Soft Tissue Sarcomas of the Chest Wall
Next Article in Special Issue
The Future of Immunotherapy-Based Combination Therapy in Metastatic Renal Cell Carcinoma
Previous Article in Journal
Histone 2A Family Member J Drives Mesenchymal Transition and Temozolomide Resistance in Glioblastoma Multiforme
Previous Article in Special Issue
Molecular Genetics of Renal Cell Tumors: A Practical Diagnostic Approach
Open AccessReview

Sarcomatoid Dedifferentiation in Renal Cell Carcinoma: From Novel Molecular Insights to New Clinical Opportunities

1
Department of Oncology, Institut de Cancérologie de Strasbourg, Hôpitaux Universitaires de Strasbourg, Université de Strasbourg, 67200 Strasbourg, France
2
Department of Cancer and Functional Genomics, Institute of Genetics and Molecular and Cellular Biology, CNRS/INSERM/UNISTRA, 67400 Illkirch, France
3
Department of Pathology, Centre Hospitalier Universitaire Régional de Strasbourg, 67200 Strasbourg, France
4
Department of Urology, Centre Hospitalier Universitaire Régional de Strasbourg, 67000 Strasbourg, France
5
Department of Cancer Medicine, Gustave Roussy, 94800 Villejuif, France
*
Author to whom correspondence should be addressed.
These authors contributed equally to this paper.
These authors contributed equally to this paper.
Cancers 2020, 12(1), 99; https://doi.org/10.3390/cancers12010099
Received: 29 October 2019 / Revised: 19 December 2019 / Accepted: 20 December 2019 / Published: 31 December 2019
(This article belongs to the Special Issue Renal Cell Carcinoma)
Sarcomatoid features in renal cell carcinoma (RCC) have long been associated with dismal prognosis and poor response to therapy, while biological mechanisms underpinning sarcomatoid dedifferentiation remained obscure. Several efforts have been conducted to break down the molecular profile of sarcomatoid RCC and investigate different targeted therapeutic approaches. Mutations enriched for in sarcomatoid RCC involve, notably, TP53, BAP1, cell cycle, and chromatin-remodeling genes. The immunological landscape of these tumors is also gradually being uncovered, showing frequent expression of programmed cell death ligand-1 (PD-L1) and high levels of tumor-infiltrating lymphocytes. These features may be major determinants for the activity of immune checkpoint inhibitors in this population, which has been confirmed by retrospective studies and subgroup analyses of large randomized phase 3 trials. Combinations based on PD-1/PD-L1 inhibition have demonstrated response rates and complete responses in >50% and >10% of patients in the first-line metastatic setting, respectively, with median overall survival exceeding two years. This remarkable improvement in outcomes effectively establishes immune checkpoint inhibitor combinations as a new standard of care in patients with sarcomatoid RCC. New research fields, including epigenetic regulations and tumor–microenvironment interactions, may further sharpen understanding of sarcomatoid RCC and advance therapeutic developments. View Full-Text
Keywords: renal cell carcinoma; sarcomatoid; immunotherapy renal cell carcinoma; sarcomatoid; immunotherapy
Show Figures

Figure 1

MDPI and ACS Style

Debien, V.; Thouvenin, J.; Lindner, V.; Barthélémy, P.; Lang, H.; Flippot, R.; Malouf, G.G. Sarcomatoid Dedifferentiation in Renal Cell Carcinoma: From Novel Molecular Insights to New Clinical Opportunities. Cancers 2020, 12, 99. https://doi.org/10.3390/cancers12010099

AMA Style

Debien V, Thouvenin J, Lindner V, Barthélémy P, Lang H, Flippot R, Malouf GG. Sarcomatoid Dedifferentiation in Renal Cell Carcinoma: From Novel Molecular Insights to New Clinical Opportunities. Cancers. 2020; 12(1):99. https://doi.org/10.3390/cancers12010099

Chicago/Turabian Style

Debien, Véronique; Thouvenin, Jonathan; Lindner, Véronique; Barthélémy, Philippe; Lang, Hervé; Flippot, Ronan; Malouf, Gabriel G. 2020. "Sarcomatoid Dedifferentiation in Renal Cell Carcinoma: From Novel Molecular Insights to New Clinical Opportunities" Cancers 12, no. 1: 99. https://doi.org/10.3390/cancers12010099

Find Other Styles
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Access Map by Country/Region

1
Search more from Scilit
 
Search
Back to TopTop