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European Spine Journal

, Volume 27, Supplement 3, pp 440–445 | Cite as

Intradural cervical chordoma with diffuse spinal leptomeningeal spread: case report and review of the literature

  • Jing Zhang
  • Chuan-ping Gao
  • Xue-jun Liu
  • Wen-Jian Xu
Case Report

Abstract

Purpose

Chordoma is a low-grade malignant bone tumor derived from embryonic notochord remnants along the axial skeleton. About 50% of chordomas occur in the sacral vertebrae and 35% in the skull base. Most chordomas are extradural and cause extensive bone destruction. Intradural spinal tumors without bone involvement are rare.

Methods

We herein describe the clinical features of a patient with a chordoma as well as the imaging and pathological manifestations of the tumor.

Results

We encountered an unusual presentation of a C6 and C7 spinal intradural chordoma in a 23-year-old man. He presented with a 5-day history of discomfort over the lumbosacral region. Magnetic resonance imaging and enhanced scanning of the cervical spine showed an intradural soft tissue mass at C6 and C7 and linear enhancement of the spinal meninges. The tumor was excised because the patient had been previously misdiagnosed with an intraspinal neurogenic tumor with spinal meningitis. Postoperative pathological examination confirmed the diagnosis of chordoma. On postoperative day 7, the patient underwent brain magnetic resonance imaging because of severe headache. The images showed multiple soft tissue nodules in the skull base cistern. To the best of our knowledge, this is the first case report of an entirely extraosseous and spinal intradural chordoma with diffuse spinal leptomeningeal spread. The patient died 2 months postoperatively.

Conclusions

An intradural spinal chordoma is difficult to distinguish from a neurogenic tumor by imaging. When the lesion is dumbbell-shaped, it is easily misdiagnosed as a schwannoma. In the present case, the tumor was intradural and located at the level of the C6 and C7 vertebrae. Preoperative diagnosis was difficult, and the final diagnosis required pathological examination.

Keywords

Chordoma Notochord Intradural Spinal leptomeningeal spread Magnetic resonance imaging 

Notes

Funding

No funds were received in support of this work.

Compliance with ethical standards

Conflict of interest

None of the authors has any potential conflict of interest.

Informed consent

The patient and/or his family were informed that data from the case would be submitted for publication and gave their consent.

References

  1. 1.
    Mirra JM, Rocca CD, Nelson SD, Chordoma MF (2004) Tumours of the soft tissue and bone. In: Fletcher CDM, Unni KK, Mertens F (eds) World Health Organization Classification of Tumours. Pathology and genetics. IARC Press, Lyon, pp 316–317Google Scholar
  2. 2.
    Katayama Y, Tsubokawa T, Hirasawa T, Takahata T, Nemoto N (1991) Intradural extraosseous chordoma in the foramen magnumregion. Case report. J Neurosurg 75:976–979CrossRefPubMedGoogle Scholar
  3. 3.
    Chambers PW, Schwinn CP (1979) Chordoma. A clinicopathologic study of metastasis. Am J Clin Pathol 72:765–776CrossRefPubMedGoogle Scholar
  4. 4.
    Mehnert F, Beschorner R, Küker W, Hahn U, Nägele T (2004) Retroclival ecchordosis physaliphora: MR imaging and review of the literature. Am J Neuroradiol 25:1851–1855PubMedGoogle Scholar
  5. 5.
    Luschka H (1856) Die altersveränderungen der Zwischenwirbelknorpel. Virchows Arch 9:311–327CrossRefGoogle Scholar
  6. 6.
    Wolfe JT, Scheithauer BW (1987) “Intradural chordoma” or “giant ecchordosis physaliphora”? Report of two cases. Clin Neuropathol 6:98–103PubMedGoogle Scholar
  7. 7.
    Warnick RE, Raisanen J, Kaczmar T Jr, Davis RL, Prados MD (1991) Intradural chordoma of the tentorium cerebelli. Case report. J Neurosurg 74:508–511CrossRefPubMedGoogle Scholar
  8. 8.
    Korinth M, Schönrock L, Mayfrank L, Gilsbach JM (1999) Primary intradural pontocerebellar chordoma metastasizing in the subarachnoid spinal canal. Zentralbl Neurochir 60:146–150PubMedGoogle Scholar
  9. 9.
    Nishigaya K, Kaneko M, Ohashi Y, Nukui H (1998) Intradural retroclival chordoma without bone involvement: no tumor regrowth 5 years after operation. Case report. J Neurosurg 88:764–768CrossRefPubMedGoogle Scholar
  10. 10.
    Ciarpaglini R, Pasquini E, Mazzatenta D, Ambrosini-Spaltro A, Sciarretta V, Frank G (2009) Intradural clival chordoma and ecchordosis physaliphora; a challenging differential diagnosis: case report. Neurosurgery 64:E387–E388.  https://doi.org/10.1227/01.NEU.0000337064.57270.F0 CrossRefPubMedGoogle Scholar
  11. 11.
    Ramiro J, Ferreras B, Pérez Calvo JM, Zafra A (1986) Thoracic intradural chordoma. Neurosurgery 64:571–572Google Scholar
  12. 12.
    Steenberghs J, Kiekens C, Menten J, Monstrey J (2002) Intradural chordoma without bone involvement. Case report and review of the literature. J Neurosurg 97:94–97PubMedGoogle Scholar
  13. 13.
    Badwal L C S, Pal L, Basu A, Saxena S (2006) Multiple synchronous spinal extra-osseous intradural chordomas: is it a distinct entity? British J Neurosurg 20:99–99CrossRefGoogle Scholar
  14. 14.
    Vaz RM, Pereira JC, Ramos U, Cruz CR (1995) Intradural cervical chordoma without bone involvement. J Neurosurg 82:650–653CrossRefPubMedGoogle Scholar
  15. 15.
    Gelabert-Gonzále M, Pintos-Martínez E, Caparrini-Escondrillas A, Martínez-Rumbo R (1999) Intradural cervical chordoma. Case report. J Neurol Sci 43:159–162Google Scholar
  16. 16.
    Gunnarsson T, Leszniewski W, Bak J, Davidsson L (2001) An intradural cervical chordoma mimicking a neurinoma. Case illustration. J Neurosurg 95:144PubMedGoogle Scholar
  17. 17.
    Sumi M, Yamamoto T, Kitagawa A, Ikeda M (2001) Case illustration. J Neurosurg 94:336PubMedGoogle Scholar
  18. 18.
    Bayar M A, Erdem Y, Tanyel O, Ozturk K, Buharali Z (2002) Spinal chordoma of the terminal filum. Case report. J Neurosurg 96:236PubMedGoogle Scholar
  19. 19.
    Bergmann M, Abdalla Y, Neubauer U, Schildhaus H U, Probst-Cousin S (2010) Primary intradural chordoma: report on three cases and review of the literature. Clin Neuropathol 29:169CrossRefPubMedGoogle Scholar
  20. 20.
    Kawanabe Y, Ueda S, Sasaki N, Hoshimaru M (2014) Simultaneous discovery of cranial and spinal intradural chordomas: case report. Neurol Med Chir 54:930–935CrossRefGoogle Scholar
  21. 21.
    Uda T, Ohata K, Takami T, Hara M (2006) An intradural skull base chordoma presenting with acute intratumoral hemorrhage. Neurol India 54:306–307CrossRefPubMedGoogle Scholar
  22. 22.
    Masui K, Kawai S, Yonezawa T, Fujimoto K, Nishi N (2006) Intradural retroclival chordoma without bone involvement—case report. Neurol Med Chir (Tokyo) 46:552–555CrossRefGoogle Scholar
  23. 23.
    Katayama Y, Tsubokawa T, Hirasawa T, Takahata T, Nemoto N (1991) Intradural extraosseous chordoma in the foramen magnum region. J Neurosurg 75:976–979CrossRefPubMedGoogle Scholar
  24. 24.
    AlOtaibi F, Guiot MC, Muanza T, Di Maio D (2014). Giant petroclival primary intradural chordoma: case report and systematic review of the literature. J Neurol Surg Rep 75:e150–e169CrossRefGoogle Scholar
  25. 25.
    Wang L, Wu Z, Tian K, Li G, Zhang J (2013) Clinical and pathological features of intradural retroclival chordoma. World Neurosurg 82:791–798.  https://doi.org/10.1016/j.wneu.2012.12.037 CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Radiology DepartmentThe Affiliated Hospital of Qingdao UniversityQingdaoChina

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