Systemic Therapy for chordoma publications - Trial results and case series/case reports

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

2	Phase 2 Trial	Immune Checkpoint Inhibitor	Pembrolizumab	Keytruda	PD1	34	3 PR, median PFS 6.6 months	Blay et al. J Clin Oncol 2021
3	Phase 2 Trial	Therapeutic Vaccine	GI-6301	Tarmogen	Brachyury	24	No difference in ORR for vaccine vs. placebo	DeMaria et al. J Clin Oncol 2020
4	Phase 2 Trial	Immune Checkpoint Inhibitor	Durvalumab + tremelimumab	Imfinzi + (no brand name)	PD1 + CTLA4	5	1 PR	Somaiah et al. J Clin Oncol 2020
5	Phase 2 Trial	Immune Checkpoint Inhibitor	Pembrolizumab	Keytruda	PD1	12	1 PR, median PFS 5.7 months	Blay et al. Ann Oncol. 2019
6	Phase 2 Trial	Targeted Therapy	Dasatinib	Sprycel	PDGRF, c-KIT	32	median PFS 6.3 months	Schuetze et al. Cancer 2016
7	Phase 2 Trial	Targeted Therapy	Sorafenib	Nexavar	multiple RTK	27	1 PR, 73% SD (at 9 months)	Bompas et al. Ann Oncol. 2015
8	Phase 2 Trial	Targeted Therapy	Lapatinib	Tykerb	EGFR	18	13 SD	Stacchiotti et al. Ann Oncol. 2013
9	Phase 2 Trial	Targeted Therapy	Imatinib	Gleevec	PDGFR	50	1 PR, 70% SD, median PFS 9.2 months	Stacchiotti et al. Journal of Clinical Oncology, 2012
10	Phase 2 Trial	Targeted Therapy	Imatinib + everolimus	Gleevec + Afinitor	PDGFR + mTOR	43	1 PR, 86% SD	Stacchiotti et al. Cancer, 2018
11	Phase 2 Trial	Targeted Therapy	Sunitinib	Sutent	multiple RTK	9	4 SD	George et al. J Clin Oncol. 2009
12	Phase 2 Trial	Cytotoxic Therapy	Rubitecan	Orathecin	N/A	15	1 PR	Chugh et al. J Clin Oncol. 2005
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14	Phase 1 Trial	Therapeutic Vaccine	MVA-BN-Brachyury		Brachyury	10	1 PR, 2 SD	DeMaria et al. J Clin Oncol 2021
15	Phase 1 Trial	Targeted Therapy + Radiation	Nilotinib	Tasigna	PDGFR	23	median PFS 58.15 months	Cote et al. Int J Radiat Oncol Biol Phys 2018
16	Phase 1 Trial	Targeted Therapy	Tazemetostat	Tazverik	EZH2	2	1 CR, 1 PR	Chi et al. 2017 (Abstract)
17	Phase 1 Trial	Therapeutic Vaccine	GI-6301	Tarmogen	Brachyury	10	median PFS 8.3 months	Heery et al. Cancer Immunol Res. 2015
18	Phase 1 Trial	Targeted Therapy + Cytotoxic Therapy	Imatinib mesyate + metronomic cyclophosphamide	Gleevec + Cytoxan	PDGFR	7	median PFS 10.2 months	Adenis et al. British Journal of Cancer, 2013
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20	Case Series	Targeted Therapy	Erlotinib	Tarceva	EGFR	31	4 PR, 14 SD, median PFS 6.2 months	Mir et al. J Clin Oncol 2021
21	Case Series	Targeted Therapy	Imatinib	Gleevec	PDGFR	62	3 PR, 43 SD	Lebellec et al. Eur J Cancer 2017
22	Case Series	Targeted Therapy	Erlotinib	Tarceva	EGFR	5	1 PR, 4 SD	Lebellec et al. Eur J Cancer 2017
23	Case Series	Targeted Therapy	Sorafenib	Nexavar	multiple RTK	11	1PR, 9 SD	Lebellec et al. Eur J Cancer 2017
24	Case Series	Targeted Therapy	Pazopanib + Sunitinib	Votrient + Sutent	multiple RTK	5	median PFS 8.5 months	Lipplaa et al. Clin Sarcoma Res 2016
25	Case Series	Targeted Therapy	Imatinib	Gleevec	PDGFR	46	34 SD	Hindi et al. Euro J Cancer 2015
26	Case Series	Targeted Therapy	Imatinib	Gleevec	PDGFR	2	2 SD	Rohatgi et al. Eurasian J Med 2015
27	Case Series	Targeted Therapy	Erlotinib + Bevacizumab	Tarceva + Avastin	EGFR, VEGF	3	3 SD	Asklund et. al. Acta Oncol. 2014 Jan
28	Case Series	Targeted Therapy	Imatinib	Gleevec	PDGFR	17	1 SD	Ferraresi et al. BMC Cancer. 2010
29	Case Series	Targeted Therapy	Imatinib + Sirolimus	Gleevec + Rapamune	PDGFR + mTOR	9	8 SD	Stacchiotti et al. Ann Oncol. 2009
30	Case Series	Targeted Therapy	Imatinib	Gleevec	PDGFR	3	1 PR, 2 SD	Geoerger et al. Eur J Cancer 2009
31	Case Series	Targeted Therapy	Imatinib	Gleevec	PDGFR	6	4 symptomatic improvement	Casali et al. Cancer 2004
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33	Case Report	Targeted Therapy	Pazopanib	Votrient	VEGFR/PDGFR	1	1 SD	Ribeiro et al. Case Rep Oncol Med. 2018
34	Case Report	Targeted Therapy	Temsirolimus	Torisel	mTOR	1	1 SD	Lebellec et al. Eur J Cancer 2017
35	Case Report	Immune Checkpoint Inhibitor	Nivolumab	Opdivo	PD1	1	1 PR	Migliorini et al. OncoImmunology 2017
36	Case Report	Immune Checkpoint Inhibitor	Pembrolizumab	Keytruda	PD1	1	1 PR	Migliorini et al. OncoImmunology 2017
37	Case Report	Targeted Therapy	Pazopanib	Votrient	VEGFR/PDGFR	1	1PR	Jagersberg et al. Acta Neurochir. 2017
38	Case Report	Therapeutic Vaccine	MVX-ONCO-1		N/A	1	1 PR	Migliorini et al. OncoImmunology 2017
39	Case Report	Targeted Therapy	Erlotinib + Linsitinib	Tarceva + Linsitinib	EGFR + IGF1R	1	1 PR	Aleksic et al. Frontiers in Oncology 2017
40	Case Report	Targeted Therapy	Erlotinib	Tarceva	EGFR	1	1 PR	Houessinon et al. Case Reports Oncol. 2015
41	Case Report	Targeted Therapy	Sorafenib	Nexavar	multiple RTK	1	1 SD	Svoboda et al. J Am Acad Dermatol. 2012
42	Case Report	Cytoxoic Therapy	Vincristine + clophosphamide +doxorubicin + etoposide + ifosfamide			1	1 CR (durable for 3+ years)	Al-Rahawan MM et al. Pediatr Blood Cancer 2012
43	Case Report	Targeted Therapy	Thalidomide	Thalomide		1	1 PR	Chay et al. J Clin Oncol. 2011
44	Case Report	Targeted Therapy	Cetuximab + Gefitinib	Erbitux + Iressa	EGFR	1	1 PR	Linden et al. Acta Oncol. 2009
45	Case Report	Targeted Therapy	Erlotinib	Tarceva	EGFR	1	1 PR	Singhal et al. Anticancer Drugs. 2009
46	Case Report	Targeted Therapy	Cetuximab + Gefitinib	Erbitux + Iressa	EGFR	1	1 PR	Hof et al. Onkologie. 2006
47	Case Report	Targeted Therapy + Cytotoxic Therapy	Thalidomide + liposomal doxorubicin	Thalomid + Doxil		1	1 SD	Schönegger et al. Anticancer Drugs. 2005
48	Case Report	Cytotoxic Therapy	Ifosfamide + doxorubicin			1	1 PR	Scimeca eta al. J Pediatr Hematol Oncol. 1996
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