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Dedifferentiated Chordoma

MedGen UID:
220430
Concept ID:
C1266174
Neoplastic Process
Synonyms: Dedifferentiated chordoma; Sarcomatoid Chordoma
SNOMED CT: Dedifferentiated chordoma (128785008)

Definition

A high-grade malignant bone tumor arising from the remnants of the notochord. It is characterized by a lobulated growth pattern, myxoid stroma formation, the presence of physaliphorous cells, and a sarcomatous component. [from NCI]

Professional guidelines

PubMed

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
Evangelisti G, Fiore MR, Bandiera S, Barbanti Brodano G, Terzi S, Girolami M, Pipola V, Righi A, Nanni C, Fanti S, Ghermandi R, Molinelli S, Orecchia R, Boriani S, Gasbarrini A
Eur Rev Med Pharmacol Sci 2019 May;23(9):4002-4009. doi: 10.26355/eurrev_201905_17830. PMID: 31115029

Recent clinical studies

Etiology

Vuong HG, Le MK, Nguyen TPX, Eschbacher K
World Neurosurg 2023 May;173:208-217.e7. Epub 2023 Feb 16 doi: 10.1016/j.wneu.2023.02.062. PMID: 36804481
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
Meng T, Yin H, Li B, Li Z, Xu W, Zhou W, Cheng M, Wang J, Zhou L, Yang X, Liu T, Yan W, Song D, Xiao J
Neuro Oncol 2015 May;17(5):725-32. Epub 2014 Dec 8 doi: 10.1093/neuonc/nou331. PMID: 25488908Free PMC Article
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
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

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
Vuong HG, Le MK, Nguyen TPX, Eschbacher K
World Neurosurg 2023 May;173:208-217.e7. Epub 2023 Feb 16 doi: 10.1016/j.wneu.2023.02.062. PMID: 36804481
Ulici V, Hart J
Arch Pathol Lab Med 2022 Mar 1;146(3):386-395. doi: 10.5858/arpa.2020-0258-RA. PMID: 34319396
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
Hung YP, Diaz-Perez JA, Cote GM, Wejde J, Schwab JH, Nardi V, Chebib IA, Deshpande V, Selig MK, Bredella MA, Rosenberg AE, Nielsen GP
Am J Surg Pathol 2020 Sep;44(9):1213-1223. doi: 10.1097/PAS.0000000000001501. PMID: 32427623

Therapy

Evangelisti G, Fiore MR, Bandiera S, Barbanti Brodano G, Terzi S, Girolami M, Pipola V, Righi A, Nanni C, Fanti S, Ghermandi R, Molinelli S, Orecchia R, Boriani S, Gasbarrini A
Eur Rev Med Pharmacol Sci 2019 May;23(9):4002-4009. doi: 10.26355/eurrev_201905_17830. PMID: 31115029
Ikeda H, Honjo J, Sakurai H, Mitsuhashi N, Fukuda T, Niibe H
Radiat Med 1997 Mar-Apr;15(2):109-11. PMID: 9192436
Fleming GF, Heimann PS, Stephens JK, Simon MA, Ferguson MK, Benjamin RS, Samuels BL
Cancer 1993 Aug 1;72(3):714-8. doi: 10.1002/1097-0142(19930801)72:3<714::aid-cncr2820720314>3.0.co;2-1. PMID: 8334623

Prognosis

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
Vuong HG, Le MK, Nguyen TPX, Eschbacher K
World Neurosurg 2023 May;173:208-217.e7. Epub 2023 Feb 16 doi: 10.1016/j.wneu.2023.02.062. PMID: 36804481
Ulici V, Hart J
Arch Pathol Lab Med 2022 Mar 1;146(3):386-395. doi: 10.5858/arpa.2020-0258-RA. PMID: 34319396
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
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

Clinical prediction guides

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
Evangelisti G, Fiore MR, Bandiera S, Barbanti Brodano G, Terzi S, Girolami M, Pipola V, Righi A, Nanni C, Fanti S, Ghermandi R, Molinelli S, Orecchia R, Boriani S, Gasbarrini A
Eur Rev Med Pharmacol Sci 2019 May;23(9):4002-4009. doi: 10.26355/eurrev_201905_17830. PMID: 31115029
Meng T, Yin H, Li B, Li Z, Xu W, Zhou W, Cheng M, Wang J, Zhou L, Yang X, Liu T, Yan W, Song D, Xiao J
Neuro Oncol 2015 May;17(5):725-32. Epub 2014 Dec 8 doi: 10.1093/neuonc/nou331. PMID: 25488908Free PMC Article
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
Crapanzano JP, Ali SZ, Ginsberg MS, Zakowski MF
Cancer 2001 Feb 25;93(1):40-51. PMID: 11241265

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