TY - JOUR
T1 - Prognostic relevance of CCN3 in Ewing sarcoma
AU - Perbal, Bernard
AU - Lazar, Noureddine
AU - Zambelli, Diana
AU - Lopez-Guerrero, Jose Antonio
AU - Llombart-Bosch, Antonio
AU - Scotlandi, Katia
AU - Picci, Piero
PY - 2009/10
Y1 - 2009/10
N2 - Ewing sarcoma is a highly aggressive malignant bone tumor occurring preferentially in children and young adults. At present, only clinical features, such as patient age, presence of clinically evident metastases at diagnosis, and poor response to neoadjuvant chemotherapy, are widely accepted as prognostic indicators in Ewing sarcoma. In this study, we assessed the prognostic value of CCN3 (Nov), a matricellular protein that play crucial roles in bone formation. Polyclonal antibodies directed against each of the different CCN3 modules were used to identify variant CCN3 proteins in tumors and to draw potential relationships between the expression of these variants and the outcome of patients with Ewing sarcoma. Our results confirmed that expression of the full-length CCN3 in Ewing sarcoma is associated to a worse prognostic. Furthermore, we report a possible relationship between the expression of a CCN3 protein lacking an internal module (von Willebrand factor type C) and sensitivity to radiotherapy. We hypothesize that the increased level of variant CCN3 in the tumor cells reduces their tumorigenic potential and results in better outcome.
AB - Ewing sarcoma is a highly aggressive malignant bone tumor occurring preferentially in children and young adults. At present, only clinical features, such as patient age, presence of clinically evident metastases at diagnosis, and poor response to neoadjuvant chemotherapy, are widely accepted as prognostic indicators in Ewing sarcoma. In this study, we assessed the prognostic value of CCN3 (Nov), a matricellular protein that play crucial roles in bone formation. Polyclonal antibodies directed against each of the different CCN3 modules were used to identify variant CCN3 proteins in tumors and to draw potential relationships between the expression of these variants and the outcome of patients with Ewing sarcoma. Our results confirmed that expression of the full-length CCN3 in Ewing sarcoma is associated to a worse prognostic. Furthermore, we report a possible relationship between the expression of a CCN3 protein lacking an internal module (von Willebrand factor type C) and sensitivity to radiotherapy. We hypothesize that the increased level of variant CCN3 in the tumor cells reduces their tumorigenic potential and results in better outcome.
KW - CCN3
KW - Ewing sarcoma
KW - Matrix
KW - Prognostic
UR - http://www.scopus.com/inward/record.url?scp=69949121664&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=69949121664&partnerID=8YFLogxK
U2 - 10.1016/j.humpath.2009.05.008
DO - 10.1016/j.humpath.2009.05.008
M3 - Article
C2 - 19695675
AN - SCOPUS:69949121664
VL - 40
SP - 1479
EP - 1486
JO - Human Pathology
JF - Human Pathology
SN - 0046-8177
IS - 10
ER -