Resumen
In this review, we aim to summarize the current clinical and biological knowledge for patients affected by a rare soft-tissue sarcoma, the CIC-rearranged sarcoma, to highlight novel treatment perspectives and to remark the need of innovative clinical trials. CIC-rearranged sarcoma is still entrapped in unspecific therapeutic regimens which result in poor prognosis and high incidence of recurrence. The comprehension of the biology of this tumor and the exact mechanisms of action of its molecular markers are mandatory to identify specific cancer vulnerabilities to exploit for therapy. Clinical research for rare tumors is complicated by the difficulties in patients? recruitment and the lack of interest of Big Pharma in the development of new treatments. An intriguing perspective is given by the identification of CIC and its targets dysregulation as a common determinant of different tumor types, which might fasten the development of effective mechanism-based therapies.