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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

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVConventional Chordoma

Inherited neuroendocrine tumor
- Chordoma
  - Conventional Chordoma
    - Chondroid chordoma
      - Clivus chondroid chordoma
      - Sellar Chondroid 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: 31338785 Free 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

See all (2)

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: 36192442 Free 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

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

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

See all (8)

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

See all (26)

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: 34392582 Free 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

See all (3)

Prognosis

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

Chondroid chordoma versus low-grade chondrosarcoma of the base of the skull: can immunohistochemistry resolve the controversy?

Ishida T, Dorfman HD
J Neurooncol 1994;18(3):199-206. doi: 10.1007/BF01328954. PMID: 7525890

See all (19)

Clinical prediction guides

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

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

Chordoma: a cytologic study with histologic and radiologic correlation.

Crapanzano JP, Ali SZ, Ginsberg MS, Zakowski MF
Cancer 2001 Feb 25;93(1):40-51. PMID: 11241265

Intraoperative squash and touch cytology of chondroid chordoma of the skull base. Report of a case with immunocytochemical and immunohistochemical studies.

Nabeshima K, Sato S, Sameshima T, Goya T, Ohno A, Hinoura Y, Koono M
Acta Cytol 1997 May-Jun;41(3):913-8. doi: 10.1159/000332728. PMID: 9167726

See all (6)