Correlation of Ezrin Expression Pattern and Clinical Outcomes in Ewing Sarcoma
Thomas Cash,
Hong Yin,
Courtney McCracken,
Zhi Geng,
Steven G. DuBois,
Bahig M. Shehata,
Thomas A. Olson,
Howard M. Katzenstein,
Cynthia Wetmore
Affiliations
Thomas Cash
Department of Pediatrics, Emory University, Children’s Healthcare of Atlanta, Health Sciences Research Building, Brumley Bridge, 3rd Floor, W-350, 1760 Haygood Drive, Atlanta, GA 30322, USA
Hong Yin
Department of Pathology, Emory University, Children’s Healthcare of Atlanta, 1405 Clifton Road NE, Atlanta, GA 30322, USA
Courtney McCracken
Department of Pediatrics, Emory University, Children’s Healthcare of Atlanta, Health Sciences Research Building, Brumley Bridge, 4th Floor, W-440-B, 1760 Haygood Drive, Atlanta, GA 30322, USA
Zhi Geng
Department of Pediatrics, Emory University, Children’s Healthcare of Atlanta, Health Sciences Research Building, Brumley Bridge, 4th Floor, W-440-B, 1760 Haygood Drive, Atlanta, GA 30322, USA
Steven G. DuBois
Dana-Farber/Boston Children’s Cancer and Blood Disorders Center and Harvard Medical School, 450 Brookline Avenue, Boston, MA 02215, USA
Bahig M. Shehata
Department of Pathology, Emory University, Children’s Healthcare of Atlanta, 1405 Clifton Road NE, Atlanta, GA 30322, USA
Thomas A. Olson
Department of Pediatrics, Emory University, Children’s Healthcare of Atlanta, 1405 Clifton Road NE, Atlanta, GA 30322, USA
Howard M. Katzenstein
Department of Pediatrics, Vanderbilt University, Monroe Carell Jr. Children’s Hospital at Vanderbilt, 2220 Pierce Avenue, 396c Preston Research Building, Nashville, TN 37232, USA
Cynthia Wetmore
Department of Pediatrics, Emory University, Children’s Healthcare of Atlanta, Health Sciences Research Building, Brumley Bridge, 4th Floor, W-470, 1760 Haygood Drive, Atlanta, GA 30322, USA
Background. Ezrin is a membrane-cytoskeleton linker protein that has been associated with metastasis and poor outcomes in osteosarcoma and high-grade soft tissue sarcomas. The prognostic value of ezrin expression in Ewing sarcoma is unknown. Methods. The relationship between ezrin expression and outcome was analyzed in a cohort of 53 newly diagnosed Ewing sarcoma patients treated between 2000 and 2011. The intensity and proportion of cells with ezrin immunoreactivity were assessed in diagnostic tumor tissue using a semiquantitative scoring system to yield intensity and positivity scores for each tumor. Results. Ezrin expression was detected in 72% (38/53) of tumor samples. The proportion of patients with metastatic disease was equal in the positive and negative ezrin expression groups. There was no significant difference in the 5-year event-free survival (EFS) between patients with positive versus negative ezrin expression. Patients whose tumor sample showed high ezrin intensity had significantly better 5-year EFS when compared to patients with low/no ezrin intensity (78% versus 55%; P=0.03). Conclusions. Ezrin expression can be detected in the majority of Ewing sarcoma tumor samples. Intense ezrin expression may be correlated with a favorable outcome; however further investigation with a larger cohort is needed to validate this finding.
