Medical treatment of advanced chordomas

Imatinib and everolimus in patients with progressing advanced chordoma: A phase 2 clinical study

“…1 Even after optimal treatment of the primary tumor, most patients with chordoma have local and/or metastatic recurrences that cannot be cured. [1][2][3] In 2010, we reported the results of the molecular analysis of tumor samples from 22 naive chordomas showing an involvement of the mammalian target of rapamycin (mTOR) pathway in most patients. In recent years, molecular therapies (including imatinib, sorafenib, and EGFR inhibitors) have been introduced but have had limited impact on disease outcome.…”

“…In recent years, molecular therapies (including imatinib, sorafenib, and EGFR inhibitors) have been introduced but have had limited impact on disease outcome. [1][2][3] In 2010, we reported the results of the molecular analysis of tumor samples from 22 naive chordomas showing an involvement of the mammalian target of rapamycin (mTOR) pathway in most patients. 4 Afterward, we explored the antitumor effect of imatinib plus sirolimus in 10 chordoma patients with secondary resistance to imatinib, observing 1 and 6 partial responses (PRs) by RECIST and Choi criteria, respectively.…”

Development and Validation of a 6-miRNA Prognostic Signature in Spinal Chordoma

“…Despite the great advance in oncology field, the current treatment of choice for chordoma is still limited. Chordoma is resistance to traditional chemotherapy or radiotherapy (3). Therefore, radical surgical excision constitutes the most important therapeutic approach for chordoma, which has been reported to provide the optimal long-term disease control when combined with postoperative adjuvant radiation (4,5).…”

Advancement of PI3 Kinase Inhibitor Combination Therapies for PI3K-Aberrant Chordoma

“…Unfortunately, these tumors have also been shown to be relatively resistant to traditional cytotoxic chemotherapies. 2 Imatinib, a targeted inhibitor of BCR-ABL, c-KIT, and PDGFR, has shown some benefit in phase-II clinical trials and currently is the most commonly used systemic therapy. [2][3][4] Despite aggressive treatment regimens, recurrence rates are reported to be as high as 68% with minimal improvement in median survival over the past four decades.…”