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Conventional Chordoma

MedGen UID:
1791577
•Concept ID:
C5555136
•
Neoplastic Process

Definition

A malignant bone tumor arising from the remnants of the fetal notochord. It is characterized by the presence of large epithelioid cells with clear to light eosinophilic cytoplasm. Some of the cells are large with bubbly, vacuolated cytoplasm (physaliphorous cells). The cells form cords and nests that are embedded within an extracellular myxoid matrix. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Treatment of dedifferentiated chordoma: a retrospective study from a large volume cancer center.
Nachwalter RN, Rothrock RJ, Katsoulakis E, Gounder MM, Boland PJ, Bilsky MH, Laufer I, Schmitt AM, Yamada Y, Higginson DS
J Neurooncol 2019 Sep;144(2):369-376. Epub 2019 Jul 23 doi: 10.1007/s11060-019-03239-3. PMID: 31338785Free PMC Article
Medical treatment of advanced chordomas.
Colia V, Stacchiotti S
Eur J Cancer 2017 Sep;83:220-228. Epub 2017 Jul 25 doi: 10.1016/j.ejca.2017.06.038. PMID: 28750274

Recent clinical studies

Etiology

Differences in stromal component of chordoma are associated with contrast enhancement in MRI and differential gene expression in RNA sequencing.
Park M, Park I, Hong CK, Kim SH, Cha YJ
Sci Rep 2022 Oct 3;12(1):16504. doi: 10.1038/s41598-022-20787-3. PMID: 36192442Free PMC Article
Predict overall survival of spinal conventional chordoma: Development and assessment of a new predictive nomogram.
Lin K, Song K, Wang S, Jiang L, Wang H, Dong J
Clin Neurol Neurosurg 2020 Oct;197:106174. Epub 2020 Aug 22 doi: 10.1016/j.clineuro.2020.106174. PMID: 32889324
Treatment of dedifferentiated chordoma: a retrospective study from a large volume cancer center.
Nachwalter RN, Rothrock RJ, Katsoulakis E, Gounder MM, Boland PJ, Bilsky MH, Laufer I, Schmitt AM, Yamada Y, Higginson DS
J Neurooncol 2019 Sep;144(2):369-376. Epub 2019 Jul 23 doi: 10.1007/s11060-019-03239-3. PMID: 31338785Free PMC Article
Prognostic factors in the operative management of dedifferentiated sacral chordomas.
Kayani B, Sewell MD, Hanna SA, Saifuddin A, Aston W, Pollock R, Skinner J, Molloy S, Briggs TW
Neurosurgery 2014 Sep;75(3):269-75; discussion 275. doi: 10.1227/NEU.0000000000000423. PMID: 24867206
Clinicopathological characteristics of chordoma: an institutional experience and a review of the literature.
Makhdoomi R, Ramzan A, Khursheed N, Bhat S, Baba K, Mohsin R, Basharat M, Yameen B, Ahmad R, Iqbal L, Maqbool M, Ajaz H, Farhat M
Turk Neurosurg 2013;23(6):700-6. doi: 10.5137/1019-5149.JTN.5941-12.3. PMID: 24310451

Diagnosis

Squash smear and fine needle aspiration features of conventional chordoma.
Gajdzis P, Klijanienko J, Gajdzis M
Cytopathology 2023 Sep;34(5):503-506. Epub 2023 Mar 13 doi: 10.1111/cyt.13225. PMID: 36840426
Chordoma.
Ulici V, Hart J
Arch Pathol Lab Med 2022 Mar 1;146(3):386-395. doi: 10.5858/arpa.2020-0258-RA. PMID: 34319396
Notochordal Tumors.
Tirabosco R, O'Donnell P, Flanagan AM
Surg Pathol Clin 2021 Dec;14(4):619-643. Epub 2021 Jul 28 doi: 10.1016/j.path.2021.06.006. PMID: 34742484
Predict overall survival of spinal conventional chordoma: Development and assessment of a new predictive nomogram.
Lin K, Song K, Wang S, Jiang L, Wang H, Dong J
Clin Neurol Neurosurg 2020 Oct;197:106174. Epub 2020 Aug 22 doi: 10.1016/j.clineuro.2020.106174. PMID: 32889324
Molecular characteristics of poorly differentiated chordoma.
Shih AR, Chebib I, Deshpande V, Dickson BC, Iafrate AJ, Nielsen GP
Genes Chromosomes Cancer 2019 Nov;58(11):804-808. Epub 2019 Jun 13 doi: 10.1002/gcc.22782. PMID: 31135077

Therapy

Recurrent loss of chromosome 22 and SMARCB1 deletion in extra-axial chordoma: A clinicopathological and molecular analysis.
Wen X, Cimera R, Aryeequaye R, Abhinta M, Athanasian E, Healey J, Fabbri N, Boland P, Zhang Y, Hameed M
Genes Chromosomes Cancer 2021 Dec;60(12):796-807. Epub 2021 Aug 26 doi: 10.1002/gcc.22992. PMID: 34392582Free PMC Article
Predict overall survival of spinal conventional chordoma: Development and assessment of a new predictive nomogram.
Lin K, Song K, Wang S, Jiang L, Wang H, Dong J
Clin Neurol Neurosurg 2020 Oct;197:106174. Epub 2020 Aug 22 doi: 10.1016/j.clineuro.2020.106174. PMID: 32889324
Medical treatment of advanced chordomas.
Colia V, Stacchiotti S
Eur J Cancer 2017 Sep;83:220-228. Epub 2017 Jul 25 doi: 10.1016/j.ejca.2017.06.038. PMID: 28750274

Prognosis

Incidence and centralization of chordoma in the Netherlands: A nationwide study between 1991 and 2020.
Lipplaa A, van der Wal RJP, Krol ADG, Peul WC, Bovée JVMG, Gelderblom H
Cancer Epidemiol 2024 Apr;89:102527. Epub 2024 Jan 25 doi: 10.1016/j.canep.2024.102527. PMID: 38277716
Chordoma.
Ulici V, Hart J
Arch Pathol Lab Med 2022 Mar 1;146(3):386-395. doi: 10.5858/arpa.2020-0258-RA. PMID: 34319396
Notochordal Tumors.
Tirabosco R, O'Donnell P, Flanagan AM
Surg Pathol Clin 2021 Dec;14(4):619-643. Epub 2021 Jul 28 doi: 10.1016/j.path.2021.06.006. PMID: 34742484
Predict overall survival of spinal conventional chordoma: Development and assessment of a new predictive nomogram.
Lin K, Song K, Wang S, Jiang L, Wang H, Dong J
Clin Neurol Neurosurg 2020 Oct;197:106174. Epub 2020 Aug 22 doi: 10.1016/j.clineuro.2020.106174. PMID: 32889324
Molecular characteristics of poorly differentiated chordoma.
Shih AR, Chebib I, Deshpande V, Dickson BC, Iafrate AJ, Nielsen GP
Genes Chromosomes Cancer 2019 Nov;58(11):804-808. Epub 2019 Jun 13 doi: 10.1002/gcc.22782. PMID: 31135077

Clinical prediction guides

Incidence and centralization of chordoma in the Netherlands: A nationwide study between 1991 and 2020.
Lipplaa A, van der Wal RJP, Krol ADG, Peul WC, Bovée JVMG, Gelderblom H
Cancer Epidemiol 2024 Apr;89:102527. Epub 2024 Jan 25 doi: 10.1016/j.canep.2024.102527. PMID: 38277716
Squash smear and fine needle aspiration features of conventional chordoma.
Gajdzis P, Klijanienko J, Gajdzis M
Cytopathology 2023 Sep;34(5):503-506. Epub 2023 Mar 13 doi: 10.1111/cyt.13225. PMID: 36840426
Predict overall survival of spinal conventional chordoma: Development and assessment of a new predictive nomogram.
Lin K, Song K, Wang S, Jiang L, Wang H, Dong J
Clin Neurol Neurosurg 2020 Oct;197:106174. Epub 2020 Aug 22 doi: 10.1016/j.clineuro.2020.106174. PMID: 32889324
Lumbar vertebral chordoma arising from an intraosseous benign notochordal cell tumour: radiological findings and histopathological description with a good clinical outcome.
Nishiguchi T, Mochizuki K, Tsujio T, Nishita T, Inoue Y
Br J Radiol 2010 Mar;83(987):e49-53. doi: 10.1259/bjr/63846600. PMID: 20197427Free PMC Article
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

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