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Review
. 2018 Aug 27;12(1):239.
doi: 10.1186/s13256-018-1784-y.

Chordoma: a case series and review of the literature

Ozkan Alan  1 , Tugba Akin Telli  2 , Ozlem Ercelep  2 , Eda Tanrikulu Simsek  2 , Tugba Basoglu Tuylu  2 , Aydan Mutis  3 , Rahib Hasanov  2 , Serap Kaya  2 , Nalan Akgül Babacan  2 , Faysal Dane  2 , Perran Fulden Yumuk  2
Affiliations
Review

Chordoma: a case series and review of the literature

Ozkan Alan et al. J Med Case Rep. .

Abstract

Background: Chordoma is a rare malignant tumor of the skull base and axial skeleton, with an incidence of less than 0.1/100,000 per year. Patients with advanced chordoma have a poor prognosis due to locoregional recurrence with infiltration and destruction of surrounding bone and soft tissue. Cytotoxic chemotherapy or other systemic therapies have not been proven to be effective for these diseases. Therefore, several molecularly targeted therapies have been proposed as potentially beneficial, including tyrosine kinase inhibitors such as imatinib, sorafenib, lapatinib, and others.

Case presentation: We present three cases of advanced chordoma treated with molecular targeted therapies: a 52-year-old Caucasian man, a 72-year-old Caucasian woman, and a 38-year-old Caucasian woman.

Conclusions: Chordoma has few systemic treatment options and they have limited benefit. Randomized trials with large patient numbers are unfeasible in this rare disease. Targeted therapy might be a reasonable alternative treatment for chordoma. Still, new treatment strategies are needed for this rare disease.

Keywords: Advanced chordoma; Rare disease; Targeted therapy; Tyrosine kinases.

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Conflict of interest statement

Ethics approval and consent to participate

Not applicable.

Consent for publication

Written informed consent was obtained from the patients for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Competing interests

The authors declare that they have no competing interests.

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Figures

Fig. 1
Fig. 1
Magnetic resonance image of patient 3 treated with imatinib (progressive disease 25 months)
Fig. 2
Fig. 2
Magnetic resonance image of patient 3 treated with sunitinib (progressive disease 6 months)
See this image and copyright information in PMC

References

    1. Stiller CA, Trama A, Serraino D, Rossi S, Navarro C, Chirlaque MD, Casali PG, RARECARE Working Group Descriptive epidemiology of sarcomas in Europe: report from the RARECARE project. Eur J Cancer. 2013;49(3):684–695. doi: 10.1016/j.ejca.2012.09.011. - DOI - PubMed
    1. Fletcher CDM, Bridge JA, Hogendoorn P, Mertens F, editors. WHO Classification of Tumours of Soft Tissue and Bone. Fourth Edition. Lyon: IARC Press; 2013. p. 328–9.
    1. Stacchiotti S, Sommer J, Chordoma Global Consensus G Building a global consensus approach to chordoma: a position paper from the medical and patient community. Lancet Oncol. 2015;16:e71–e83. doi: 10.1016/S1470-2045(14)71190-8. - DOI - PubMed
    1. Moojen WA, Vleggeert-Lankamp CL, Krol AD, Dijkstra SP. Long-term results: adjuvant radiotherapy in en bloc resection of sacrococcygeal chordoma is advisable. Spine. 2011;36:E656–E661. doi: 10.1097/BRS.0b013e3181f8d1f3. - DOI - PubMed
    1. DeLaney TF, Liebsch NJ, Pedlow FX, Adams J, Dean S, Yeap BY, McManus P, Rosenberg AE, Nielsen GP, Harmon DC, Spiro IJ, Raskin KA, Suit HD, Yoon SS, Hornicek FJ. Phase II study of high-dose photon/proton radiotherapy in the management of spine sarcomas. Int J Radiat Oncol Biol Phys. 2009;74:732–739. doi: 10.1016/j.ijrobp.2008.08.058. - DOI - PMC - PubMed

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