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Chondroid chordoma

MedGen UID:
224768
•Concept ID:
C1266173
•
Neoplastic Process
Synonyms: Chondroid Chordoma; chondroid chordoma; chondroid chordoma (morphologic abnormality)
SNOMED CT: Chondroid chordoma (128784007)
 
Monarch Initiative: MONDO:0006145

Definition

A slow-growing malignant bone tumor arising from the remnants of the notochord and occurring in the base of the skull. The tumor is characterized by a lobulated growth pattern, myxoid stroma formation, and the presence of physaliphorous cells and cartilage. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChondroid chordoma

Professional guidelines

PubMed

Chondroblastic osteosarcoma: Cytomorphologic characteristics and differential diagnosis on FNA.
VandenBussche CJ, Sathiyamoorthy S, Wakely PE Jr, Ali SZ
Cancer Cytopathol 2016 Jul;124(7):493-500. Epub 2016 Mar 21 doi: 10.1002/cncy.21715. PMID: 26999748
Differential diagnosis of dumbbell lesions associated with spinal neural foraminal widening: imaging features.
Kivrak AS, Koc O, Emlik D, Kiresi D, Odev K, Kalkan E
Eur J Radiol 2009 Jul;71(1):29-41. Epub 2008 May 16 doi: 10.1016/j.ejrad.2008.03.020. PMID: 18485652
Differential diagnosis of chordoma, chondroid, and ependymal tumors as aided by anti-intermediate filament antibodies.
Miettinen M, Lehto VP, Dahl D, Virtanen I
Am J Pathol 1983 Aug;112(2):160-9. PMID: 6192722Free PMC Article

Recent clinical studies

Etiology

Population-based survival analysis of primary spinal chordoma in the US from 2000 to 2020.
Agner KE, Larkins MC
J Neurooncol 2024 Nov;170(2):397-405. Epub 2024 Sep 27 doi: 10.1007/s11060-024-04807-y. PMID: 39331221Free PMC Article
Clival chordoma in a young male patient: a case report.
Zribi A, Nasr SB, Khemir A, Gargouri F, Abdallah IB, Msakni I, Fendri S, Balti M, Laabidi B, Haddaoui A
Pan Afr Med J 2020;37:59. Epub 2020 Sep 15 doi: 10.11604/pamj.2020.37.59.24836. PMID: 33209186Free PMC Article
Endoscopic transnasal surgery of clival lesions: our experience.
Marchioni D, Musumeci A, Fabbris C, De Rossi S, Soloperto D
Eur Arch Otorhinolaryngol 2018 May;275(5):1149-1156. Epub 2018 Mar 8 doi: 10.1007/s00405-018-4928-1. PMID: 29520496
Skull base chondroid chordoma: atypical case manifesting as intratumoral hemorrhage and literature review.
Tsutsumi S, Akiba C, Suzuki T, Nakanishi H, Izumi H, Yasumoto Y, Ito M
Clin Neuroradiol 2014 Dec;24(4):313-20. Epub 2014 Jul 29 doi: 10.1007/s00062-014-0321-7. PMID: 25070287
Adjuvant radiation therapy and chondroid chordoma subtype are associated with a lower tumor recurrence rate of cranial chordoma.
Jian BJ, Bloch OG, Yang I, Han SJ, Aranda D, Tihan T, Parsa AT
J Neurooncol 2010 May;98(1):101-8. Epub 2009 Dec 2 doi: 10.1007/s11060-009-0068-1. PMID: 19953297

Diagnosis

Population-based survival analysis of primary spinal chordoma in the US from 2000 to 2020.
Agner KE, Larkins MC
J Neurooncol 2024 Nov;170(2):397-405. Epub 2024 Sep 27 doi: 10.1007/s11060-024-04807-y. PMID: 39331221Free PMC Article
Clinicopathologic characteristics of poorly differentiated chordoma.
Shih AR, Cote GM, Chebib I, Choy E, DeLaney T, Deshpande V, Hornicek FJ, Miao R, Schwab JH, Nielsen GP, Chen YL
Mod Pathol 2018 Aug;31(8):1237-1245. Epub 2018 Feb 26 doi: 10.1038/s41379-018-0002-1. PMID: 29483606
Chondrosarcomas of the head and neck.
Coca-Pelaz A, Rodrigo JP, Triantafyllou A, Hunt JL, Fernández-Miranda JC, Strojan P, de Bree R, Rinaldo A, Takes RP, Ferlito A
Eur Arch Otorhinolaryngol 2014 Oct;271(10):2601-9. Epub 2013 Nov 10 doi: 10.1007/s00405-013-2807-3. PMID: 24213203
Chondroid chordoma. A hyalinized chordoma without cartilaginous differentiation.
Jeffrey PB, Biava CG, Davis RL
Am J Clin Pathol 1995 Mar;103(3):271-9. doi: 10.1093/ajcp/103.3.271. PMID: 7532910
Chondroid chordoma.
Mierau GW, Weeks DA
Ultrastruct Pathol 1987;11(5-6):731-7. doi: 10.3109/01913128709048460. PMID: 3686707

Therapy

Chondroid chordoma in an atypical location.
Royo Crespo I, Rivas de Andrés JJ, Embún Flor R, Cortés Franco S
Arch Bronconeumol 2013 Nov;49(11):491-3. Epub 2013 Jun 18 doi: 10.1016/j.arbres.2013.03.006. PMID: 23791243
Chondroid chordoma of the skull base: immunohistochemical and ultrastructural study of two cases with special reference to microtubules within rough-surfaced endoplasmic reticulum.
Shintaku M, Maeno K, Okabe H
Med Mol Morphol 2010 Dec;43(4):241-5. Epub 2011 Jan 26 doi: 10.1007/s00795-010-0509-0. PMID: 21267702
The 'incidentaloma' of the pituitary gland. Is neurosurgery required?
Reincke M, Allolio B, Saeger W, Menzel J, Winkelmann W
JAMA 1990 May 23-30;263(20):2772-6. PMID: 2332920

Prognosis

Population-based survival analysis of primary spinal chordoma in the US from 2000 to 2020.
Agner KE, Larkins MC
J Neurooncol 2024 Nov;170(2):397-405. Epub 2024 Sep 27 doi: 10.1007/s11060-024-04807-y. PMID: 39331221Free PMC Article
Clival chordoma in a young male patient: a case report.
Zribi A, Nasr SB, Khemir A, Gargouri F, Abdallah IB, Msakni I, Fendri S, Balti M, Laabidi B, Haddaoui A
Pan Afr Med J 2020;37:59. Epub 2020 Sep 15 doi: 10.11604/pamj.2020.37.59.24836. PMID: 33209186Free PMC Article
Endoscopic transnasal surgery of clival lesions: our experience.
Marchioni D, Musumeci A, Fabbris C, De Rossi S, Soloperto D
Eur Arch Otorhinolaryngol 2018 May;275(5):1149-1156. Epub 2018 Mar 8 doi: 10.1007/s00405-018-4928-1. PMID: 29520496
Prognostic Factors in Skull Base Chordoma: A Systematic Literature Review and Meta-Analysis.
Zou MX, Lv GH, Zhang QS, Wang SF, Li J, Wang XB
World Neurosurg 2018 Jan;109:307-327. Epub 2017 Oct 16 doi: 10.1016/j.wneu.2017.10.010. PMID: 29045855
Chondrosarcomas of the head and neck.
Coca-Pelaz A, Rodrigo JP, Triantafyllou A, Hunt JL, Fernández-Miranda JC, Strojan P, de Bree R, Rinaldo A, Takes RP, Ferlito A
Eur Arch Otorhinolaryngol 2014 Oct;271(10):2601-9. Epub 2013 Nov 10 doi: 10.1007/s00405-013-2807-3. PMID: 24213203

Clinical prediction guides

Notochordal cell derived lesions: a 55-year casuistic analysis of 50 cases with radiologic-pathologic correlation in a tertiary referral hospital, and literature review.
Pena-Burgos EM, Torena Lerchundi N, Fuentes-Sánchez J, Tapia-Viñe M, Fernández-Baíllo N, Pozo-Kreilinger JJ
Eur Spine J 2024 Sep;33(9):3315-3323. Epub 2024 Jul 24 doi: 10.1007/s00586-024-08419-y. PMID: 39048841
Prognostic Factors in Skull Base Chordoma: A Systematic Literature Review and Meta-Analysis.
Zou MX, Lv GH, Zhang QS, Wang SF, Li J, Wang XB
World Neurosurg 2018 Jan;109:307-327. Epub 2017 Oct 16 doi: 10.1016/j.wneu.2017.10.010. PMID: 29045855
Chordomas of the upper cervical spine: clinical characteristics and surgical management of a series of 21 patients.
Zhou H, Jiang L, Wei F, Yu M, Wu F, Liu X, Liu Z, Dang G
Chin Med J (Engl) 2014;127(15):2759-64. PMID: 25146609
Clinical and histopathologic features of chordomas in children and young adults.
Ridenour RV 3rd, Ahrens WA, Folpe AL, Miller DV
Pediatr Dev Pathol 2010 Jan-Feb;13(1):9-17. doi: 10.2350/09-01-0584.1. PMID: 19348512
Chordomas of the skull base: follow-up review and prognostic factors.
Colli BO, Al-Mefty O
Neurosurg Focus 2001 Mar 15;10(3):E1. doi: 10.3171/foc.2001.10.3.2. PMID: 16734401

Recent systematic reviews

Prognostic Factors in Skull Base Chordoma: A Systematic Literature Review and Meta-Analysis.
Zou MX, Lv GH, Zhang QS, Wang SF, Li J, Wang XB
World Neurosurg 2018 Jan;109:307-327. Epub 2017 Oct 16 doi: 10.1016/j.wneu.2017.10.010. PMID: 29045855

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