Epithelioid Sarcoma: Opportunities for Biology-Driven Targeted Therapy.
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.
Royal Marsden Hospital , London , UK.
Jonathan
J
Noujaim
Royal Marsden Hospital , London , UK.
Khin
K
Thway
Children's Cancer Therapy Development Institute , Fort Collins, CO , USA.
Zia
Z
Bajwa
Children's Cancer Therapy Development Institute , Fort Collins, CO , USA.
Ayeza
A
Bajwa
Adult and Paediatric Sarcoma Program, Tisch Cancer Institute, Mount Sinai School of Medicine , New York, NY , USA.
Robert G
RG
Maki
Royal Marsden Hospital , London , UK.
Robin L
RL
Jones
Children's Cancer Therapy Development Institute , Fort Collins, CO , USA.
Charles
C
Keller
BAF47; INI1; SMARCB1; SWI/SNF complex; epithelioid sarcoma