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Notochordal tumor

MedGen UID:
233252
•Concept ID:
C1335069
•
Neoplastic Process
Synonyms: cancer of notochord; malignant neoplasm of notochord; malignant notochord neoplasm; neoplasm of notochord; notochord cancer; notochord neoplasm; notochord tumor; notochord tumour; notochordal cancer; Notochordal Neoplasm; notochordal neoplasm; Notochordal Tumor; notochordal tumor; tumor of notochord; tumour of notochord
 
Monarch Initiative: MONDO:0002597

Definition

A bone tumor arising from the remnants of the fetal notochord. This category includes the chordoma and benign notochordal cell tumor. [from NCI]

Professional guidelines

PubMed

Chordoma: Genetics and Contemporary Management.
Desai R, Pelargos PE, Dunn IF
Int J Mol Sci 2024 May 28;25(11) doi: 10.3390/ijms25115877. PMID: 38892063Free PMC Article
Clinical management of pediatric chordomas: a comprehensive review.
Reardon T, Marsh C, Rippe P, Ruzys D, Ayres B, Cline D, Fiani B
Acta Neurol Belg 2021 Dec;121(6):1407-1414. Epub 2021 Oct 14 doi: 10.1007/s13760-021-01821-z. PMID: 34648142
Chordoma: current concepts, management, and future directions.
Walcott BP, Nahed BV, Mohyeldin A, Coumans JV, Kahle KT, Ferreira MJ
Lancet Oncol 2012 Feb;13(2):e69-76. doi: 10.1016/S1470-2045(11)70337-0. PMID: 22300861

Recent clinical studies

Etiology

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

Diagnosis

Chordoma: analysis of 47 fine-needle aspiration biopsy, cytologic imprint, and small biopsy specimens.
Wakely PE Jr, Saoud C, Ali SZ
J Am Soc Cytopathol 2023 Jan-Feb;12(1):30-40. Epub 2022 Sep 23 doi: 10.1016/j.jasc.2022.09.005. PMID: 36270910
Chordomas: A review with emphasis on their pathophysiology, pathology, molecular biology, and genetics.
Karpathiou G, Dumollard JM, Dridi M, Dal Col P, Barral FG, Boutonnat J, Peoc'h M
Pathol Res Pract 2020 Sep;216(9):153089. Epub 2020 Jun 29 doi: 10.1016/j.prp.2020.153089. PMID: 32825957
Spontaneous tension pneumocephalus and pneumoventricle in ecchordosis physaliphora: case report of a rare presentation and review of the literature.
Ghimire P, Shapey J, Bodi I, Connor S, Thomas N, Barkas K
Br J Neurosurg 2020 Oct;34(5):537-542. Epub 2019 May 11 doi: 10.1080/02688697.2019.1594695. PMID: 31079493
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
Revisiting chordoma with brachyury, a "new age" marker: analysis of a validation study on 51 cases.
Jambhekar NA, Rekhi B, Thorat K, Dikshit R, Agrawal M, Puri A
Arch Pathol Lab Med 2010 Aug;134(8):1181-7. doi: 10.5858/2009-0476-OA.1. PMID: 20670140

Prognosis

Chordomas: A review with emphasis on their pathophysiology, pathology, molecular biology, and genetics.
Karpathiou G, Dumollard JM, Dridi M, Dal Col P, Barral FG, Boutonnat J, Peoc'h M
Pathol Res Pract 2020 Sep;216(9):153089. Epub 2020 Jun 29 doi: 10.1016/j.prp.2020.153089. PMID: 32825957

Clinical prediction guides

Chordomas: A review with emphasis on their pathophysiology, pathology, molecular biology, and genetics.
Karpathiou G, Dumollard JM, Dridi M, Dal Col P, Barral FG, Boutonnat J, Peoc'h M
Pathol Res Pract 2020 Sep;216(9):153089. Epub 2020 Jun 29 doi: 10.1016/j.prp.2020.153089. PMID: 32825957

Recent systematic reviews

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

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