Systemic Therapy for chordoma publications

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1	Therapeutic Class	Drug Name	Brand Name	Target	Evidence	Patients	Results	Reference

2	Targeted Therapy	Dasatinib	Sprycel	PDGRF, c-KIT	Phase II Trial	32	median PFS 6.3 months	Schuetze et al. Cancer 2016
3	Targeted Therapy	Sorafenib	Nexavar	multiple RTK	Phase II Trial	27	1 PR, 73% SD (at 9 months)	Bompas et al. Ann Oncol. 2015
4	Targeted Therapy	Lapatinib		EGFR	Phase II Trial	18	13 SD	Stacchiotti et al. Ann Oncol. 2013
5	Targeted Therapy	Imatinib	Gleevec	PDGFR	Phase II Trial	50	1 PR, 64% SD (at 6 months)	Stacchiotti et al. Journal of Clinical Oncology, 2012
6	Targeted Therapy	Sunitinib	Sutent	multiple RTK	Phase II Trial	9	4 SD	George et al. J Clin Oncol. 2009
7	Cytotoxic Therapy	Rubitecan	Orathecin	N/A	Phase II Trial	15	1 PR	Chugh et al. J Clin Oncol. 2005
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9	Targeted Therapy	Tazemetostat		EZH2	Phase I Trial	2	1 CR, 1 PR	Chi et al. 2017 (Abstract)
10	Therapeutic Vaccine	GI-6301	Tarmogen	Brachyury	Phase I Trial	10	median PFS 8.3 months	Heery et al. Cancer Immunol Res. 2015
11	Targeted Therapy + Cytotoxic Therapy	Imatinib mesyate + metronomic cyclophosphamide	Gleevec + Cytoxan	PDGFR	Phase I Trial	7	median PFS 10.2 months	Adenis et al. British Journal of Cancer, 2013
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13	Targeted Therapy	Pazopanib	Votrient	VEGF	Case Report	1	1 SD	Ribeiro et al. Case Rep Oncol Med. 2018
14	Immune Checkpoint Inhibitor	Nivolumab	Opdivo	PD1	Case Report	1	1 PR	Migliorini et al. OncoImmunology 2017
15	Immune Checkpoint Inhibitor	Pembrolizumab	Keytruda	PD1	Case Report	1	1 PR	Migliorini et al. OncoImmunology 2017
16	Therapeutic Vaccine	MVX-ONCO-1		N/A	Case Report	1	1 PR	Migliorini et al. OncoImmunology 2017
17	Targeted Therapy	Erlotinib + Linsitinib	Tarceva + Linsitinib	EGFR + IGF1R	Case Report	1	1 PR	Aleksic et al. Frontiers in Oncology 2017
18	Targeted Therapy	Pazopanib + Sunitinib	Votrient + Sutent	multiple RTK	Case Series	5	median PFS 8.5 months	Lipplaa et al. Clin Sarcoma Res 2016
19	Targeted Therapy	Erlotinib	Tarceva	EGFR	Case Report	1	1 PR	Houessinon et al. Case Reports Oncol. 2015
20	Targeted Therapy	Imatinib	Gleevec	PDGFR	Case Series	46	34 SD	Hindi et al. Euro J Cancer 2015
21	Targeted Therapy	Imatinib	Gleevec	PDGFR	Case Series	2	2 SD	Rohatgi et al. Eurasian J Med 2015
22	Targeted Therapy	Erlotinib + Bevacizumab	Tarceva + Avastin	EGFR, VEGF	Case Series	3	3 SD	Asklund et. al. Acta Oncol. 2014 Jan
23	Cytoxoic Therapy	Vincristine + clophosphamide +doxorubicin + etoposide + ifosfamide			Case Report	1	1 CR (durable for 3+ years)	Al-Rahawan MM et al. Pediatr Blood Cancer 2012
24	Targeted Therapy	Thalidomide	Thalomide		Case Report	1	1 PR	Chay et al. J Clin Oncol. 2011
25	Targeted Therapy	Imatinib	Gleevec	PDGFR	Case Series	17	1 SD	Ferraresi et al. BMC Cancer. 2010
26	Targeted Therapy	Imatinib + Sirolimus	Gleevec + Rapamune	PDGFR + mTOR	Case Series	9	8 SD	Stacchiotti et al. Ann Oncol. 2009
27	Targeted Therapy	Cetuximab + Gefitinib	Erbitux + Iressa	EGFR	Case Report	1	1 PR	Linden et al. Acta Oncol. 2009
28	Targeted Therapy	Erlotinib	Tarceva	EGFR	Case Report	1	1 PR	Singhal et al. Anticancer Drugs. 2009
29	Targeted Therapy	Cetuximab + Gefitinib	Erbitux + Iressa	EGFR	Case Report	1	1 PR	Hof et al. Onkologie. 2006
30	Targeted Therapy + Cytotoxic Therapy	Thalidomide + liposomal doxorubicin	Thalomid + Doxil		Case Report	1	1 SD	Schönegger et al. Anticancer Drugs. 2005
31	Targeted Therapy	Imatinib	Gleevec	PDGFR	Case Series	6	4 symptomatic improvment	Casali et al. Cancer 2004
32	Cytotoxic Therapy	Ifosfamide + doxorubicin			Case Report	1	1 PR	Scimeca eta al. J Pediatr Hematol Oncol. 1996
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