Current Approaches in the Diagnosis, Treatment and Management of Sacral Chordomas

Year 2023, Volume: 76 Issue: 3, 234 - 237, 30.06.2024

Murat Zaimoğlu , Eray Serhat Aktan Özgür Kesici Baran Can Alpergin Siavash Haşimoğlu Emre Bahir Mete , Özgür Orhan , Ümit Eroğlu Yusuf Şükrü Çağlar

Abstract

Objectives: Sacral chordoma (SC) is a rare, aggressive tumor with a high recurrence rate and originates from notochordal remnants. SC is difficult to differentiate with its non-specific symptoms such as lower back pain. Surgical treatment and radiotherapy are first-line treatments. Resection with wide margins is vital for the prevention of recurrence.

Materials and Methods: Nine patients diagnosed with SC between January 2014 and December 2021 were retrospectively analyzed. All adult
patients with SC were included in the study. Pediatric population was excluded from the study. Pathology, radiology and surgical records of the
patients’ were used. Mean age was 53.2.

Results: Two out of 9 patients died during follow-up and 5 patients had local recurrence. No metastasis was observed.

Conclusion: Current evidence shows that surgical treatment is necessary for SC’s treatment. Radiotherapy is also an important aspect of the
treatment. Existing evidence should be meta-analyzed for a better understanding of SC’s treatment and outcomes.

Keywords

Chordoma , Sacrum , Recurrence

Ethical Statement

Ethical approval was obtained from Ankara University School of Medicine Human Research Ethics Committee (approval no: 2022000106-1, date: 09.03.2022).

Supporting Institution

-

Project Number

-

Thanks

-

Sakral Kordoma Tanı ve Tedavisinde Güncel Yaklaşımlar

Abstract

Amaç: Sakral kordoma (SK), yüksek nüks oranına sahip nadir ve agresif bir tümördür ve notokord kalıntılarından kaynaklanır. SK, alt sırt ağrısı gibi
spesifik olmayan semptomlarıyla diğer patolojilerden ayırt edilmesi zor bir durumdur. Cerrahi tedavi ve radyoterapi birinci basamak tedavilerdir.
Geniş sınırlarla rezeksiyon, nüksün önlenmesi için öneme sahiptir.

Gereç ve Yöntem: Ocak 2014 ile Aralık 2021 tarihleri arasında SK tanısı konmuş 9 hasta retrospektif olarak analiz edildi. Tüm erişkin hastalar
çalışmaya dahil edildi. Pediatrik popülasyon çalışma dışı bırakıldı. Hastaların patoloji, radyoloji ve cerrahi kayıtları kullanıldı. Ortalama yaş 53,2 idi.

Bulgular: Dokuz hastanın takip süreci boyunca 2’si hayatını kaybetti ve 5 hastada lokal nüks görüldü. Metastaz gözlenmedi.

Sonuç: Mevcut kanıtlar, SK tedavisi için cerrahi müdahalenin gerekliliğini göstermektedir. Radyoterapi de tedavinin önemli bir kısmını oluşturmaktadır.
SK tedavisinin ve sonuçlarının daha iyi anlaşılması için mevcut kanıtlarla yapılacak meta-analize ihtiyaç duyulmaktadır.

Keywords

Kordoma , Sacrum , Nüks

Ethical Statement

-

Supporting Institution

-

Project Number

-

Thanks

-

References

• 1. Stiller CA, Trama A, Serraino D, et al. Descriptive epidemiology of sarcomas in Europe: report from the RARECARE project. Eur J Cancer. 2013;49:684- 695.
• 2. Farsad K, Kattapuram SV, Sacknoff R, et al. Sacral chordoma. Radiographics. 2009;29:1525-1530.
• 3. Pillai S, Govender S. Sacral chordoma : A review of literature. J Orthop. 2018;15:679-684.
• 4. Walcott BP, Nahed BV, Mohyeldin A, et al. Chordoma: current concepts, management, and future directions. Lancet Oncol. 2012;13:e69-e76.
• 5. Yang XR, Ng D, Alcorta DA, et al. T (brachyury) gene duplication confers major susceptibility to familial chordoma. Nat Genet. 2009;41:1176-1178.
• 6. Chen KW, Yang HL, Kandimalla Y, et al. Review of current treatment of sacral chordoma. Orthop Surg. 2009;1:238-244.
• 7. Fourney DR, Gokaslan ZL. Current management of sacral chordoma. Neurosurg Focus. 2003;15:E9.
• 8. Zhang K, Chen H, Wu G, et al. High expression of SPHK1 in sacral chordoma and association with patients’ poor prognosis. Med Oncol. 2014;31:247.
• 9. Ruosi C, Colella G, Di Donato SL, et al. Surgical treatment of sacral chordoma: survival and prognostic factors. Eur Spine J. 2015;24(Suppl 7):912-917.
• 10. Hulen CA, Temple HT, Fox WP, et al. Oncologic and functional outcome following sacrectomy for sacral chordoma. J Bone Joint Surg Am. 2006;88:1532-1539.
• 11. Hug EB, Fitzek MM, Liebsch NJ, et al. Locally challenging osteo- and chondrogenic tumors of the axial skeleton: results of combined proton and photon radiation therapy using three-dimensional treatment planning. Int J Radiat Oncol Biol Phys. 1995;31:467-476.
• 12. Zabel-du Bois A, Nikoghosyan A, Schwahofer A, et al. Intensity modulated radiotherapy in the management of sacral chordoma in primary versus recurrent disease. Radiother Oncol. 2010;97:408-412.
• 13. Zhou J, Yang B, Wang X, et al. Comparison of the Effectiveness of Radiotherapy with Photons and Particles for Chordoma After Surgery: A Meta-Analysis. World Neurosurg. 2018;117:46-53.
• 14. Nishida Y, Kamada T, Imai R, Tsukushi S, Yamada Y, Sugiura H, Shido Y, Wasa J, Ishiguro N. Clinical outcome of sacral chordoma with carbon ion radiotherapy compared with surgery. Int J Radiat Oncol Biol Phys. 2011;79:110-116.
• 15. IBM Corp. Released 2013. IBM SPSS Statistics for Windows, Version 22.0. Armonk, NY: IBM Corp.
• 16. Radaelli S, Stacchiotti S, Ruggieri P, et al. Sacral Chordoma: Long-term Outcome of a Large Series of Patients Surgically Treated at Two Reference Centers. Spine (Phila Pa 1976). 2016;41:1049-1057.
• 17. Demizu Y, Imai R, Kiyohara H, et al. Carbon ion radiotherapy for sacral chordoma: A retrospective nationwide multicentre study in Japan. Radiother Oncol. 2021;154:1-5.