CASE REPORT

Surgical Treatment of Thoracic Chordoma

Tratamento Cirúrgico de Cordoma Torácica

  • Matheus Felipe Henriques Brandão 1
  • Maurus Marques de Almeida Holanda 2
  • José Lopes Sousa Filho 3
  • Christian Diniz Ferreira 2
  • Daniel de Araújo Paz 3
  • Leticia Gomes Félix da Silva 1
  • Maurus Marques de Almeida Holanda Filho 4
  Views: 306
  Downloads: 7

Resumo

Cordoma é um tumor ósseo agressivo e raro que representa 1-4% dos tumores ósseos e afeta cerca de 0,1/100.000 pessoas/ano. Sua maior incidência está na região do clivus, sacro, e é menos comum na coluna dorsal. Seus achados clínicos são inicialmente inespecíficos. Assim, à medida que o tumor cresce, sintomas mais específicos - associados à compressão nervosa - tendem a aparecer. O melhor exame de imagem para visualização do cordoma e planejamento cirúrgico é a ressonância magnética. O diagnóstico confirmatório é feito por microscopia e, principalmente, por estudo imuno-histoquímico. O tratamento consiste principalmente na ressecção cirúrgica máxima e radiação específica. A recorrência é comum apesar destas modalidades de tratamento. Nosso estudo destaca um caso raro de cordoma torácico e o papel da remoção total como uma opção de tratamento eficaz.

Palavras-chave

Cordoma; Coluna; Vértebras; Corpectomia

Abstract

Chordoma is an aggressive and rare bone tumor that represents 1-4% of bone tumors and affects about 0.1/100,000 people/year. Its highest incidence is in the region of the clivus, sacrum, and is less common in the dorsal spine. Its clinical findings are initially nonspecific. Thus, as the tumor grows, more specific symptoms - associated with nerve compression - tend to appear. The best imaging exam for visualization of the chordoma and surgical planning is magnetic resonance imaging. The confirmatory diagnosis is made by microscopy and, mainly, by immunohistochemistry study. Treatment consists mainly of maximal surgical resection and specific radiation. Recurrence is common despite these treatment modalities. Our study highlights a rare case of thoracic chordoma and the role of total removal as an effective treatment option.

Keywords

Chordoma; Spine; Vertebrates; Corpectomy

References

1. Faheem M, Zeeshan Q, Ojha B, Agrawal P. Thoracic intramedullary chordoma without bone involvement: a rare clinical entity. BMJ Case Rep. 2016;2016:1-4. http://doi.org/10.1136/bcr-2016-216101. PMid:27469385.

2. Miyazawa N, Ishigame K, Kato S, Satoh Y, Shinohara T. Thoracic chordoma: review and role of FDG-PET. J Neurosurg Sci. 2008;52(4):117- 21. PMid:18946438.

3. Bisceglia M, D’Angelo VA, Guglielmi G, Ben Dor D, Pasquinelli G. Dedifferentiated chordoma of the thoracic spine with rhabdomyosarcomatous differentiation: report of a case and review of the literature. Ann Diagn Pathol. 2007;11(4):262-73. http://doi. org/10.1016/j.anndiagpath.2006.09.002. PMid:17630110.

4. Moune MY, Sayore CM, Hemama M, El Fatemi N, El Maaqili MR. Surgical management of a thoracic chordoma: a case report and literature review. Surg Neurol Int. 2022;13:63. http://doi.org/10.25259/ SNI_1227_2021. PMid:35242429.

5. Burke JF, Chan AK, Mayer RR, et al. Clamshell thoracotomy for en bloc resection of a 3-level thoracic chordoma: technical note and operative video. Neurosurg Focus. 2020;49(3):e16. http://doi. org/10.3171/2020.6.FOCUS20382. PMid:32871571.

6. Liu S, Zhou X, Song A, et al. Surgical treatment of giant chordoma in the thoracic spine combining thoracoscopic and posterior spinal surgery. Medicine. 2019;98(35):e16990. http://doi.org/10.1097/ MD.0000000000016990. PMid:31464948.

7. Hung YP, Diaz-Perez JA, Cote GM, et al. Dedifferentiated chordoma: clinicopathologic and molecular characteristics with integrative analysis. Am J Surg Pathol. 2020;44(9):1213-23. http://doi.org/10.1097/ PAS.0000000000001501. PMid:32427623.

8. Wang T, Shu S, Chen L, et al. Thoracic chordoma: an unusual presentation of the spinal tumor. Am J Med Sci. 2008;335(3):239-41. http://doi.org/10.1097/MAJ.0b013e3180ebeac3. PMid:18344701.

9. Sobash PT, Vedala K, Alfano D, Pinckard-Dover H, Muesse JL, Desikan R. A rare case of chordoma presenting as a Pancoast tumor. Rare Tumors. 2021;13:20363613211029493. http://doi. org/10.1177/20363613211029493. PMid:34276922.

10. Sciubba DM, Gokaslan ZL, Black JH 3rd, et al. 5-level spondylectomy for en Bloc resection of thoracic chordoma: case report. Neurosurgery. 2011;69(2, Suppl Operative):e248-55. PMid:21937945.

11. Pinter NK, Pfiffner TJ, Mechtler LL. Neuroimaging of spine tumors. Handb Clin Neurol. 2016;136:689-706. http://doi.org/10.1016/B978- 0-444-53486-6.00033-8. PMid:27430436.

12. Murakami H, Kawahara N, Tomita K, Demura S, Kato S, Yoshioka K. Does interruption of the artery of adamkiewicz during total en bloc spondylectomy affect neurologic function? Spine. 2010;35(22):e1187- 92. http://doi.org/10.1097/BRS.0b013e3181e215e5. PMid:20959771.

13. Denaro L, Berton A, Ciuffreda M, et al. Surgical management of chordoma: a systematic review. J Spinal Cord Med. 2020;43(6):797-812. http://doi.org/10.1080/10790268.2018.1483593. PMid:30048230.



1Faculdade de Medicina Nova Esperança – FAMENE, João Pessoa, PB, Brasil.

2Universidade Federal da Paraíba – UFPB, João Pessoa, PB, Brasil.

3Hospital Metropolitano Dom José Maria Pires, Santa Rita, PB, Brasil.

4Faculdade de Ciências Médicas da Paraíba – FCM-PB, Cabedelo, PB, Brasil.

 

Received Mar 5, 2023

Corrected May 9, 2023

Accepted June 30, 2023

JBNC  Brazilian Journal of Neurosurgery

JBNC
  •   ISSN (print version): 0103-5118
  •   e-ISSN (online version): 2446-6786
iThenticate
Open Access

Contact

Social Media

   

ABNc  Academia Brasileira de Neurocirurgia

  •   Rua da Quitanda 159 – 10º andar - Centro - CEP 20091-005 - Rio de Janeiro - RJ
  •   +55 21 2233.0323
  •    abnc@abnc.org.br

Sponsor

  • Brain4Care
  • Hospital INC
  • Strattner
  • Zeiss