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Czauderna, Piotr; Haeberle, Beate; Hiyama, Eiso; Rangaswami, Arun; Krailo, Mark; Maibach, Rudolf; Rinaldi, Eugenia; Feng, Yurong; Aronson, Daniel; Malogolowkin, Marcio; Yoshimura, Kenichi; Leuschner, Ivo; Lopez-Terrada, Dolores; Hishiki, Tomoro; Perilongo, Giorgio; Schweinitz, Dietrich von; Schmid, Irene; Watanabe, Kenichiro; Derosa, Marisa; Meyers, Rebecka (2016): The (C)under-barhildren's (H)under-barepatic tumors (I)under-barnternational (C)under-barollaboration (CHIC): Novel global rare tumor database yields new prognostic factors in hepatoblastoma and becomes a research model. In: European Journal of Cancer, Vol. 52: pp. 92-101
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Abstract

Introduction: Contemporary state-of-the-art management of cancer is increasingly defined by individualized treatment strategies. For very rare tumors, like hepatoblastoma, the development of biologic markers, and the identification of reliable prognostic risk factors for tailoring treatment, remains very challenging. The Children's Hepatic tumors International Collaboration (CHIC) is a novel international response to this challenge. Methods: Four multicenter trial groups in the world, who have performed prospective controlled studies of hepatoblastoma over the past two decades (COG;SIOPEL;GPOH;and JPLT), joined forces to form the CHIC consortium. With the support of the data management group CINECA, CHIC developed a centralized online platform where data from eight completed hepatoblastoma trials were merged to form a database of 1605 hepatoblastoma cases treated between 1988 and 2008. The resulting dataset is described and the relationships between selected patient and tumor characteristics, and risk for adverse disease outcome (event-free survival;EFS) are examined. Results: Significantly increased risk for EFS-event was noted for advanced PRETEXT group, macrovascular venous or portal involvement, contiguous extrahepatic disease, primary tumor multifocality and tumor rupture at enrollment. Higher age (>= 8 years), low AFP (< 100 ng/ml) and metastatic disease were associated with the worst outcome. Conclusion: We have identified novel prognostic factors for hepatoblastoma, as well as confirmed established factors, that will be used to develop a future common global risk stratification system. The mechanics of developing the globally accessible web-based portal, building and refining the database, and performing this first statistical analysis has laid the foundation for future collaborative efforts. This is an important step for refining of the risk based grouping and approach to future treatment stratification, thus we think our collaboration offers a template for others to follow in the study of rare tumors and diseases. (C) 2015 Elsevier Ltd. All rights reserved.