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update on disease, epidemiology, biology and medical therapies

Frezza, Anna Mariaa; Botta, Laurab; Trama, Annalisab; Dei Tos, Angelo P.c,d; Stacchiotti, Silviaa

doi: 10.1097/CCO.0000000000000502

Purpose of review Chordoma is an exceedingly rare subtype of bone sarcoma. This review aims to provide a comprehensive insight into chordoma epidemiology, and an update on the recent advances in disease, biology and medical therapies.

Recent findings The incidence of chordoma is approximately 0.08/100 000 and the 5-year overall age-adjusted relative survival is 72% in the United States and 61% in Europe. Over the last years, significant steps forwards have been done in the comprehension of chordoma complexity, with insights gained into the biology and morphology of this disease. New entities have been described and potentially druggable molecular targets identified. This is becoming all the more relevant today, as new potentially active agents are under development.

Summary Chordoma is a complex disease because of its rarity, biological heterogeneity and peculiar clinical behaviour. Despite the progress done, the outcome in this disease remains unsatisfactory and the identification of active systemic treatments remains an urgent, unmet medical need.

aDepartment of Medical Oncology, Fondazione IRCCS Istituto Nazionale Tumori

bEvaluative Epidemiology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan

cDepartment of Pathology, Treviso Regional Hospital, Treviso

dDepartment of Medicine, University of Padua School of Medicine, Padova, Italy

Correspondence to Silvia Stacchiotti, MD, Department of Medical Oncology, Fondazione IRCCS Istututo Nazionale Tumori, Via Giacomo Venezian 1, 20133 Milan, Italy. Tel: +39 02 2390 2803; fax: +39 02 2390 2804; e-mail:

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