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Benign Notochordal Cell Tumor

MedGen UID:: 886843
•Concept ID:: C4050147
•: Neoplastic Process

Synonym:

Intraosseous Benign Notochordal Cell Tumor

Definition

An intraosseous benign tumor of notochord origin that arises in the bones of the base of the skull, vertebral bodies, sacrum or coccyx. It contains vacuolated tumor cells without atypia, but lacks myxoid matrix, necrosis, and lobular architecture, which are features that characterize its malignant counterpart, chordoma. Most lesions are incidental findings. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVBenign Notochordal Cell Tumor

Disease
- Disorder by Site
  - Connective and Soft Tissue Disorder
    - Connective tissue disorder
      - Bone Neoplasm
        Bone benign neoplasm
        Benign Notochordal Cell Tumor

Recent clinical studies

Etiology

A watch, wait, and rescan approach for incidental benign-appearing notochordal lesions of the skull base.

Usher IE, Drosos E, Morsy A, Wadeson A, Laitt R, Abdulla S, Madhavan A, Halliday J, Rutherford S, King AT, Pathmanaban ON
Neurosurg Focus 2024 May;56(5):E2. doi: 10.3171/2024.2.FOCUS23912. PMID: 38691870

Symptomatic ecchordosis physaliphora of the upper clivus: an exceedingly rare entity.

Veiceschi P, Arosio AD, Agosti E, Bignami M, Pistochini A, Cerati M, Castelnuovo P, Locatelli D
Acta Neurochir (Wien) 2021 Sep;163(9):2475-2486. Epub 2021 Apr 26 doi: 10.1007/s00701-021-04857-5. PMID: 33900480

Systematic Review of Clinical, Radiologic, and Histologic Features of Benign Notochordal Cell Tumors: Implications for Patient Management.

Usher I, Flanagan AM, Choi D
World Neurosurg 2019 Oct;130:13-23. Epub 2019 Jun 14 doi: 10.1016/j.wneu.2019.06.009. PMID: 31421435

Benign notochordal cell tumor: a retrospective study of 11 cases with 13 vertebra bodies.

Ma X, Xia C, Liu D, Liu H, Wang C, Yu H
Int J Clin Exp Pathol 2014;7(7):3548-54. Epub 2014 Jun 15 PMID: 25120733 Free PMC Article

Benign notochordal lesions of the posterior clivus: retrospective review of prevalence and imaging characteristics.

Golden LD, Small JE
J Neuroimaging 2014 May-Jun;24(3):245-9. Epub 2013 Mar 6 doi: 10.1111/jon.12013. PMID: 23464492

See all (6)

Diagnosis

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

Imaging of spinal chordoma and benign notochordal cell tumor (BNCT) with radiologic pathologic correlation.

Murphey MD, Minn MJ, Contreras AL, Koeller KK, Shih RY, Inwards CY, Yamaguchi T
Skeletal Radiol 2023 Mar;52(3):349-363. Epub 2022 Sep 5 doi: 10.1007/s00256-022-04158-7. PMID: 36063190

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

Benign notochordal cell tumor: a retrospective study of 11 cases with 13 vertebra bodies.

Ma X, Xia C, Liu D, Liu H, Wang C, Yu H
Int J Clin Exp Pathol 2014;7(7):3548-54. Epub 2014 Jun 15 PMID: 25120733 Free PMC Article

Intraosseous benign notochordal cell tumor.

Amer HZ, Hameed M
Arch Pathol Lab Med 2010 Feb;134(2):283-8. doi: 10.5858/134.2.283. PMID: 20121620

See all (18)

Prognosis

A watch, wait, and rescan approach for incidental benign-appearing notochordal lesions of the skull base.

Imaging of spinal chordoma and benign notochordal cell tumor (BNCT) with radiologic pathologic correlation.

Murphey MD, Minn MJ, Contreras AL, Koeller KK, Shih RY, Inwards CY, Yamaguchi T
Skeletal Radiol 2023 Mar;52(3):349-363. Epub 2022 Sep 5 doi: 10.1007/s00256-022-04158-7. PMID: 36063190

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

Benign notochordal lesions of the posterior clivus: retrospective review of prevalence and imaging characteristics.

Golden LD, Small JE
J Neuroimaging 2014 May-Jun;24(3):245-9. Epub 2013 Mar 6 doi: 10.1111/jon.12013. PMID: 23464492

Intraosseous benign notochordal cell tumor.

Amer HZ, Hameed M
Arch Pathol Lab Med 2010 Feb;134(2):283-8. doi: 10.5858/134.2.283. PMID: 20121620

See all (6)

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.

Endoscopic Transpterygoid Approach to Meckel's Cave: Technical Considerations and Retrospective Analysis of a Clinical Series.

Ferlendis L, Bossi B, Barillot C, Leocata A, Veiceschi P, Pozzi F, Castelnuovo P, Locatelli D
Clin Neurol Neurosurg 2024 Aug;243:108382. Epub 2024 Jun 18 doi: 10.1016/j.clineuro.2024.108382. PMID: 38944020

Symptomatic ecchordosis physaliphora of the upper clivus: an exceedingly rare entity.

Atypical Notochordal Cell Tumors: A Series of Notochordal-derived Tumors That Defy Current Classification Schemes.

Carter JM, Wenger DE, Rose PS, Inwards CY
Am J Surg Pathol 2017 Jan;41(1):39-48. doi: 10.1097/PAS.0000000000000766. PMID: 27819871

See all (4)