TY - JOUR
T1 - Epithelioid sarcoma
T2 - Opportunities for biology-driven targeted therapy
AU - Noujaim, Jonathan
AU - Thway, Khin
AU - Bajwa, Zia
AU - Bajwa, Ayeza
AU - Maki, Robert G.
AU - Jones, Robin L.
AU - Keller, Charles
N1 - Publisher Copyright:
© 2015 Noujaim, Thway, Bajwa, Bajwa, Maki, Jones and Keller.
PY - 2015
Y1 - 2015
N2 - Epithelioid sarcoma (ES) is a soft tissue sarcoma of children and young adults for which the preferred treatment for localized disease is wide surgical resection. Medical management is to a great extent undefined, and therefore for patients with regional and distal metastases, the development of targeted therapies is greatly desired. In this review, we will summarize clinically relevant biomarkers (e.g., SMARCB1, CA125, dysadherin, and others) with respect to targeted therapeutic opportunities. We will also examine the role of EGFR, mTOR, and polykinase inhibitors (e.g., sunitinib) in the management of local and disseminated disease. Toward building a consortium of pharmaceutical, academic, and non-profit collaborators, we will discuss the state of resources for investigating ES with respect to cell line resources, tissue banks, and registries so that a roadmap can be developed toward effective biology-driven therapies.
AB - Epithelioid sarcoma (ES) is a soft tissue sarcoma of children and young adults for which the preferred treatment for localized disease is wide surgical resection. Medical management is to a great extent undefined, and therefore for patients with regional and distal metastases, the development of targeted therapies is greatly desired. In this review, we will summarize clinically relevant biomarkers (e.g., SMARCB1, CA125, dysadherin, and others) with respect to targeted therapeutic opportunities. We will also examine the role of EGFR, mTOR, and polykinase inhibitors (e.g., sunitinib) in the management of local and disseminated disease. Toward building a consortium of pharmaceutical, academic, and non-profit collaborators, we will discuss the state of resources for investigating ES with respect to cell line resources, tissue banks, and registries so that a roadmap can be developed toward effective biology-driven therapies.
KW - BAF47
KW - Epithelioid sarcoma
KW - INI1
KW - SMARCB1
KW - SWI/SNF complex
UR - http://www.scopus.com/inward/record.url?scp=84940982836&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84940982836&partnerID=8YFLogxK
U2 - 10.3389/fonc.2015.00186
DO - 10.3389/fonc.2015.00186
M3 - Article
AN - SCOPUS:84940982836
SN - 2234-943X
VL - 5
JO - Frontiers in Oncology
JF - Frontiers in Oncology
IS - Aug
M1 - 186
ER -