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Advanced Chordoma

MedGen UID:
1766774
•Concept ID:
C5420150
•
Neoplastic Process

Definition

A chordoma that has spread extensively to other anatomic sites or is no longer responding to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdvanced Chordoma

Professional guidelines

PubMed

Gastrointestinal stromal tumours: ESMO-EURACAN-GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up.
Casali PG, Blay JY, Abecassis N, Bajpai J, Bauer S, Biagini R, Bielack S, Bonvalot S, Boukovinas I, Bovee JVMG, Boye K, Brodowicz T, Buonadonna A, De Álava E, Dei Tos AP, Del Muro XG, Dufresne A, Eriksson M, Fedenko A, Ferraresi V, Ferrari A, Frezza AM, Gasperoni S, Gelderblom H, Gouin F, Grignani G, Haas R, Hassan AB, Hindi N, Hohenberger P, Joensuu H, Jones RL, Jungels C, Jutte P, Kasper B, Kawai A, Kopeckova K, Krákorová DA, Le Cesne A, Le Grange F, Legius E, Leithner A, Lopez-Pousa A, Martin-Broto J, Merimsky O, Messiou C, Miah AB, Mir O, Montemurro M, Morosi C, Palmerini E, Pantaleo MA, Piana R, Piperno-Neumann S, Reichardt P, Rutkowski P, Safwat AA, Sangalli C, Sbaraglia M, Scheipl S, Schöffski P, Sleijfer S, Strauss D, Strauss SJ, Hall KS, Trama A, Unk M, van de Sande MAJ, van der Graaf WTA, van Houdt WJ, Frebourg T, Gronchi A, Stacchiotti S; ESMO Guidelines Committee, EURACAN and GENTURIS. Electronic address: clinicalguidelines@esmo.org
Ann Oncol 2022 Jan;33(1):20-33. Epub 2021 Sep 21 doi: 10.1016/j.annonc.2021.09.005. PMID: 34560242
Bone sarcomas: ESMO-EURACAN-GENTURIS-ERN PaedCan Clinical Practice Guideline for diagnosis, treatment and follow-up.
Strauss SJ, Frezza AM, Abecassis N, Bajpai J, Bauer S, Biagini R, Bielack S, Blay JY, Bolle S, Bonvalot S, Boukovinas I, Bovee JVMG, Boye K, Brennan B, Brodowicz T, Buonadonna A, de Álava E, Dei Tos AP, Garcia Del Muro X, Dufresne A, Eriksson M, Fagioli F, Fedenko A, Ferraresi V, Ferrari A, Gaspar N, Gasperoni S, Gelderblom H, Gouin F, Grignani G, Gronchi A, Haas R, Hassan AB, Hecker-Nolting S, Hindi N, Hohenberger P, Joensuu H, Jones RL, Jungels C, Jutte P, Kager L, Kasper B, Kawai A, Kopeckova K, Krákorová DA, Le Cesne A, Le Grange F, Legius E, Leithner A, López Pousa A, Martin-Broto J, Merimsky O, Messiou C, Miah AB, Mir O, Montemurro M, Morland B, Morosi C, Palmerini E, Pantaleo MA, Piana R, Piperno-Neumann S, Reichardt P, Rutkowski P, Safwat AA, Sangalli C, Sbaraglia M, Scheipl S, Schöffski P, Sleijfer S, Strauss D, Sundby Hall K, Trama A, Unk M, van de Sande MAJ, van der Graaf WTA, van Houdt WJ, Frebourg T, Ladenstein R, Casali PG, Stacchiotti S; ESMO Guidelines Committee, EURACAN, GENTURIS and ERN PaedCan. Electronic address: clinicalguidelines@esmo.org
Ann Oncol 2021 Dec;32(12):1520-1536. Epub 2021 Sep 6 doi: 10.1016/j.annonc.2021.08.1995. PMID: 34500044
Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up(☆).
Gronchi A, Miah AB, Dei Tos AP, Abecassis N, Bajpai J, Bauer S, Biagini R, Bielack S, Blay JY, Bolle S, Bonvalot S, Boukovinas I, Bovee JVMG, Boye K, Brennan B, Brodowicz T, Buonadonna A, De Álava E, Del Muro XG, Dufresne A, Eriksson M, Fagioli F, Fedenko A, Ferraresi V, Ferrari A, Frezza AM, Gasperoni S, Gelderblom H, Gouin F, Grignani G, Haas R, Hassan AB, Hecker-Nolting S, Hindi N, Hohenberger P, Joensuu H, Jones RL, Jungels C, Jutte P, Kager L, Kasper B, Kawai A, Kopeckova K, Krákorová DA, Le Cesne A, Le Grange F, Legius E, Leithner A, Lopez-Pousa A, Martin-Broto J, Merimsky O, Messiou C, Mir O, Montemurro M, Morland B, Morosi C, Palmerini E, Pantaleo MA, Piana R, Piperno-Neumann S, Reichardt P, Rutkowski P, Safwat AA, Sangalli C, Sbaraglia M, Scheipl S, Schöffski P, Sleijfer S, Strauss D, Strauss S, Sundby Hall K, Trama A, Unk M, van de Sande MAJ, van der Graaf WTA, van Houdt WJ, Frebourg T, Casali PG, Stacchiotti S; ESMO Guidelines Committee, EURACAN and GENTURIS. Electronic address: clinicalguidelines@esmo.org
Ann Oncol 2021 Nov;32(11):1348-1365. Epub 2021 Jul 22 doi: 10.1016/j.annonc.2021.07.006. PMID: 34303806

Recent clinical studies

Etiology

Weekly cisplatin with or without imatinib in advanced chordoma: A retrospective case-series analysis from the Italian Rare Cancers Network.
Baldi GG, Lo Vullo S, Grignani G, Vincenzi B, Badalamenti G, Mastore M, Buonomenna C, Morosi C, Barisella M, Frezza AM, Provenzano S, Simeone N, Picozzi F, Mariani L, Casali PG, Stacchiotti S
Cancer 2022 Apr 1;128(7):1439-1448. Epub 2022 Jan 13 doi: 10.1002/cncr.34083. PMID: 35026050
Apatinib in patients with advanced chordoma: a single-arm, single-centre, phase 2 study.
Liu C, Jia Q, Wei H, Yang X, Liu T, Zhao J, Ling Y, Wang C, Yu H, Li Z, Jiao J, Wu Z, Yang C, Xiao J
Lancet Oncol 2020 Sep;21(9):1244-1252. doi: 10.1016/S1470-2045(20)30466-6. PMID: 32888455
Imatinib and everolimus in patients with progressing advanced chordoma: A phase 2 clinical study.
Stacchiotti S, Morosi C, Lo Vullo S, Casale A, Palassini E, Frezza AM, Dinoi G, Messina A, Gronchi A, Cavalleri A, Venturelli E, Morelli D, Pilotti S, Collini P, Brich S, Tamborini E, Mariani L, Casali PG
Cancer 2018 Oct 15;124(20):4056-4063. Epub 2018 Sep 14 doi: 10.1002/cncr.31685. PMID: 30216418
Advanced chordoma treated by first-line molecular targeted therapies: Outcomes and prognostic factors. A retrospective study of the French Sarcoma Group (GSF/GETO) and the Association des Neuro-Oncologues d'Expression Française (ANOCEF).
Lebellec L, Chauffert B, Blay JY, Le Cesne A, Chevreau C, Bompas E, Bertucci F, Cupissol D, Fabbro M, Saada-Bouzid E, Duffaud F, Feuvret L, Bonneville-Levard A, Bay JO, Vauleon E, Vinceneux A, Noel G, Penel N, Mir O
Eur J Cancer 2017 Jul;79:119-128. Epub 2017 May 4 doi: 10.1016/j.ejca.2017.03.037. PMID: 28478340
Imatinib in advanced chordoma: A retrospective case series analysis.
Hindi N, Casali PG, Morosi C, Messina A, Palassini E, Pilotti S, Tamborini E, Radaelli S, Gronchi A, Stacchiotti S
Eur J Cancer 2015 Nov;51(17):2609-14. Epub 2015 Aug 14 doi: 10.1016/j.ejca.2015.07.038. PMID: 26283036

Diagnosis

The role of systemic therapy in advanced skull base chordomas: overview of the current state and the MD Anderson protocol.
Banu MA, Raza SM, Amini M, Seaman S, Rubino F, Snyder R, Patel S, DeMonte F, Conley AP
Neurosurg Focus 2024 May;56(5):E15. doi: 10.3171/2024.2.FOCUS2416. PMID: 38691867
Weekly cisplatin with or without imatinib in advanced chordoma: A retrospective case-series analysis from the Italian Rare Cancers Network.
Baldi GG, Lo Vullo S, Grignani G, Vincenzi B, Badalamenti G, Mastore M, Buonomenna C, Morosi C, Barisella M, Frezza AM, Provenzano S, Simeone N, Picozzi F, Mariani L, Casali PG, Stacchiotti S
Cancer 2022 Apr 1;128(7):1439-1448. Epub 2022 Jan 13 doi: 10.1002/cncr.34083. PMID: 35026050
Afatinib Is a New Therapeutic Approach in Chordoma with a Unique Ability to Target EGFR and Brachyury.
Magnaghi P, Salom B, Cozzi L, Amboldi N, Ballinari D, Tamborini E, Gasparri F, Montagnoli A, Raddrizzani L, Somaschini A, Bosotti R, Orrenius C, Bozzi F, Pilotti S, Galvani A, Sommer J, Stacchiotti S, Isacchi A
Mol Cancer Ther 2018 Mar;17(3):603-613. Epub 2017 Dec 13 doi: 10.1158/1535-7163.MCT-17-0324. PMID: 29237806
Advanced chordoma treated by first-line molecular targeted therapies: Outcomes and prognostic factors. A retrospective study of the French Sarcoma Group (GSF/GETO) and the Association des Neuro-Oncologues d'Expression Française (ANOCEF).
Lebellec L, Chauffert B, Blay JY, Le Cesne A, Chevreau C, Bompas E, Bertucci F, Cupissol D, Fabbro M, Saada-Bouzid E, Duffaud F, Feuvret L, Bonneville-Levard A, Bay JO, Vauleon E, Vinceneux A, Noel G, Penel N, Mir O
Eur J Cancer 2017 Jul;79:119-128. Epub 2017 May 4 doi: 10.1016/j.ejca.2017.03.037. PMID: 28478340
Imatinib mesylate in chordoma.
Casali PG, Messina A, Stacchiotti S, Tamborini E, Crippa F, Gronchi A, Orlandi R, Ripamonti C, Spreafico C, Bertieri R, Bertulli R, Colecchia M, Fumagalli E, Greco A, Grosso F, Olmi P, Pierotti MA, Pilotti S
Cancer 2004 Nov 1;101(9):2086-97. doi: 10.1002/cncr.20618. PMID: 15372471

Therapy

Targeted Therapy for Chordoma: Key Molecular Signaling Pathways and the Role of Multimodal Therapy.
Akinduro OO, Suarez-Meade P, Garcia D, Brown DA, Sarabia-Estrada R, Attia S, Gokaslan ZL, Quiñones-Hinojosa A
Target Oncol 2021 May;16(3):325-337. Epub 2021 Apr 24 doi: 10.1007/s11523-021-00814-5. PMID: 33893940Free PMC Article
Apatinib in patients with advanced chordoma: a single-arm, single-centre, phase 2 study.
Liu C, Jia Q, Wei H, Yang X, Liu T, Zhao J, Ling Y, Wang C, Yu H, Li Z, Jiao J, Wu Z, Yang C, Xiao J
Lancet Oncol 2020 Sep;21(9):1244-1252. doi: 10.1016/S1470-2045(20)30466-6. PMID: 32888455
Imatinib and everolimus in patients with progressing advanced chordoma: A phase 2 clinical study.
Stacchiotti S, Morosi C, Lo Vullo S, Casale A, Palassini E, Frezza AM, Dinoi G, Messina A, Gronchi A, Cavalleri A, Venturelli E, Morelli D, Pilotti S, Collini P, Brich S, Tamborini E, Mariani L, Casali PG
Cancer 2018 Oct 15;124(20):4056-4063. Epub 2018 Sep 14 doi: 10.1002/cncr.31685. PMID: 30216418
Chordoma: a case series and review of the literature.
Alan O, Akin Telli T, Ercelep O, Tanrikulu Simsek E, Basoglu Tuylu T, Mutis A, Hasanov R, Kaya S, Akgül Babacan N, Dane F, Yumuk PF
J Med Case Rep 2018 Aug 27;12(1):239. doi: 10.1186/s13256-018-1784-y. PMID: 30145982Free PMC Article
Molecular targeted therapies in advanced or metastatic chordoma patients: facts and hypotheses.
Lebellec L, Aubert S, Zaïri F, Ryckewaert T, Chauffert B, Penel N
Crit Rev Oncol Hematol 2015 Jul;95(1):125-31. Epub 2015 Jan 30 doi: 10.1016/j.critrevonc.2015.01.010. PMID: 25682222

Prognosis

Imatinib and everolimus in patients with progressing advanced chordoma: A phase 2 clinical study.
Stacchiotti S, Morosi C, Lo Vullo S, Casale A, Palassini E, Frezza AM, Dinoi G, Messina A, Gronchi A, Cavalleri A, Venturelli E, Morelli D, Pilotti S, Collini P, Brich S, Tamborini E, Mariani L, Casali PG
Cancer 2018 Oct 15;124(20):4056-4063. Epub 2018 Sep 14 doi: 10.1002/cncr.31685. PMID: 30216418
Chordoma: a case series and review of the literature.
Alan O, Akin Telli T, Ercelep O, Tanrikulu Simsek E, Basoglu Tuylu T, Mutis A, Hasanov R, Kaya S, Akgül Babacan N, Dane F, Yumuk PF
J Med Case Rep 2018 Aug 27;12(1):239. doi: 10.1186/s13256-018-1784-y. PMID: 30145982Free PMC Article
Advanced chordoma treated by first-line molecular targeted therapies: Outcomes and prognostic factors. A retrospective study of the French Sarcoma Group (GSF/GETO) and the Association des Neuro-Oncologues d'Expression Française (ANOCEF).
Lebellec L, Chauffert B, Blay JY, Le Cesne A, Chevreau C, Bompas E, Bertucci F, Cupissol D, Fabbro M, Saada-Bouzid E, Duffaud F, Feuvret L, Bonneville-Levard A, Bay JO, Vauleon E, Vinceneux A, Noel G, Penel N, Mir O
Eur J Cancer 2017 Jul;79:119-128. Epub 2017 May 4 doi: 10.1016/j.ejca.2017.03.037. PMID: 28478340
Molecular targeted therapies in advanced or metastatic chordoma patients: facts and hypotheses.
Lebellec L, Aubert S, Zaïri F, Ryckewaert T, Chauffert B, Penel N
Crit Rev Oncol Hematol 2015 Jul;95(1):125-31. Epub 2015 Jan 30 doi: 10.1016/j.critrevonc.2015.01.010. PMID: 25682222
Imatinib mesylate in chordoma.
Casali PG, Messina A, Stacchiotti S, Tamborini E, Crippa F, Gronchi A, Orlandi R, Ripamonti C, Spreafico C, Bertieri R, Bertulli R, Colecchia M, Fumagalli E, Greco A, Grosso F, Olmi P, Pierotti MA, Pilotti S
Cancer 2004 Nov 1;101(9):2086-97. doi: 10.1002/cncr.20618. PMID: 15372471

Clinical prediction guides

Weekly cisplatin with or without imatinib in advanced chordoma: A retrospective case-series analysis from the Italian Rare Cancers Network.
Baldi GG, Lo Vullo S, Grignani G, Vincenzi B, Badalamenti G, Mastore M, Buonomenna C, Morosi C, Barisella M, Frezza AM, Provenzano S, Simeone N, Picozzi F, Mariani L, Casali PG, Stacchiotti S
Cancer 2022 Apr 1;128(7):1439-1448. Epub 2022 Jan 13 doi: 10.1002/cncr.34083. PMID: 35026050
Apatinib in patients with advanced chordoma: a single-arm, single-centre, phase 2 study.
Liu C, Jia Q, Wei H, Yang X, Liu T, Zhao J, Ling Y, Wang C, Yu H, Li Z, Jiao J, Wu Z, Yang C, Xiao J
Lancet Oncol 2020 Sep;21(9):1244-1252. doi: 10.1016/S1470-2045(20)30466-6. PMID: 32888455
Defective homologous recombination DNA repair as therapeutic target in advanced chordoma.
Gröschel S, Hübschmann D, Raimondi F, Horak P, Warsow G, Fröhlich M, Klink B, Gieldon L, Hutter B, Kleinheinz K, Bonekamp D, Marschal O, Chudasama P, Mika J, Groth M, Uhrig S, Krämer S, Heining C, Heilig CE, Richter D, Reisinger E, Pfütze K, Eils R, Wolf S, von Kalle C, Brandts C, Scholl C, Weichert W, Richter S, Bauer S, Penzel R, Schröck E, Stenzinger A, Schlenk RF, Brors B, Russell RB, Glimm H, Schlesner M, Fröhling S
Nat Commun 2019 Apr 9;10(1):1635. doi: 10.1038/s41467-019-09633-9. PMID: 30967556Free PMC Article
Phase II study of imatinib in advanced chordoma.
Stacchiotti S, Longhi A, Ferraresi V, Grignani G, Comandone A, Stupp R, Bertuzzi A, Tamborini E, Pilotti S, Messina A, Spreafico C, Gronchi A, Amore P, Vinaccia V, Casali PG
J Clin Oncol 2012 Mar 20;30(9):914-20. Epub 2012 Feb 13 doi: 10.1200/JCO.2011.35.3656. PMID: 22331945
Imatinib mesylate in chordoma.
Casali PG, Messina A, Stacchiotti S, Tamborini E, Crippa F, Gronchi A, Orlandi R, Ripamonti C, Spreafico C, Bertieri R, Bertulli R, Colecchia M, Fumagalli E, Greco A, Grosso F, Olmi P, Pierotti MA, Pilotti S
Cancer 2004 Nov 1;101(9):2086-97. doi: 10.1002/cncr.20618. PMID: 15372471

Recent systematic reviews

Targeted Therapy for Chordoma: Key Molecular Signaling Pathways and the Role of Multimodal Therapy.
Akinduro OO, Suarez-Meade P, Garcia D, Brown DA, Sarabia-Estrada R, Attia S, Gokaslan ZL, Quiñones-Hinojosa A
Target Oncol 2021 May;16(3):325-337. Epub 2021 Apr 24 doi: 10.1007/s11523-021-00814-5. PMID: 33893940Free PMC Article

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