Skip to main content

Benign Notochordal Cell Tumor

  • Chapter
Tumors and Tumor-Like Lesions of Bone
  • 4863 Accesses

Abstract

Benign notochordal tumor is a benign indolent bone tumor probably derived from vestigial notochordal rests. As it is usually asymptomatic, its true incidence is unknown but has been found in 20 % of spines in an autopsy study. Radiographs, CT, or bone scan may appear normal. MRI shows altered signal intensity. Histologically, it appears as sheet-like proliferation of bland-looking cells with well-defined boundary, resembling fatty marrow at low magnification. Foci of chordoma may be found next to the lesion, suggesting histogenetic relationship. It is immunohistochemically positive for S100, epithelial markers, and brachyury. Surgery is only indicated in large lesions and for differential diagnosis.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 219.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 279.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Recommended Reading

  • Adamek D, Malec M, Grabska N, Krygowska-Wajs A, Galazka K. Ecchordosis physaliphora – a case report and a review of notochord-derived lesions. Neurol Neurochir Pol. 2011;45:169–73.

    PubMed  Google Scholar 

  • Alkan O, Yildirim T, Kizilkilic O, Tan M, Cekinmez M. A case of ecchordosis physaliphora presenting with an intratumoral hemorrhage. Turk Neurosurg. 2009;19:293–6.

    PubMed  Google Scholar 

  • Amer HZ, Hameed M. Intraosseous benign notochordal cell tumor. Arch Pathol Lab Med. 2010;134:283–8.

    PubMed  Google Scholar 

  • Cha ST, Jarrahy R, Yong WH, Eby T, Shahinian HK. A rare symptomatic presentation of ecchordosis physaliphora and unique endoscope-assisted surgical management. Minim Invasive Neurosurg. 2002;45:36–40.

    Article  CAS  PubMed  Google Scholar 

  • Deshpande V, Nielsen GP, Rosenthal DI, Rosenberg AE. Intraosseous benign notochord cell tumors (BNCT): further evidence supporting a relationship to chordoma. Am J Surg Pathol. 2007;31:1573–7.

    Article  PubMed  Google Scholar 

  • Golden LD, Small JE. Benign notochordal lesions of the posterior clivus: retrospective review of prevalence and imaging characteristics. J Neuroimaging. 2013;23:314–6.

    Article  Google Scholar 

  • Haasper C, Langer F, Rosenthal H, et al. Coccydynia due to a benign notochordal cell tumor. Spine (Phila Pa 1976). 2007;32:E394–6.

    Article  Google Scholar 

  • Iorgulescu JB, Laufer I, Hameed M, et al. Benign notochordal cell tumors of the spine: natural history of 8 patients with histologically confirmed lesions. Neurosurgery. 2013;73:411–6.

    Article  PubMed  Google Scholar 

  • Kikuchi Y, Yamaguchi T, Kishi H, et al. Pulmonary tumor with notochordal differentiation: report of 2 cases suggestive of benign notochordal cell tumor of extraosseous origin. Am J Surg Pathol. 2011;35:1158–64.

    Article  PubMed  Google Scholar 

  • Kyriakos M. Benign notochordal lesions of the axial skeleton: a review and current appraisal. Skelet Radiol. 2011;40:1141–52.

    Article  Google Scholar 

  • Lalam R, Cassar-Pullicino VN, McClure J, Singh J. Entrapped intralesional marrow: a hitherto undescribed imaging feature of benign notochordal cell tumour. Skelet Radiol. 2012;41:725–31.

    Article  Google Scholar 

  • Lee FY, Wen MC, Wang J. Extraosseous benign notochordal cell tumor presenting as bilateral pulmonary nodules. Hum Pathol. 2013;44:1447–51.

    Article  PubMed  Google Scholar 

  • Matsubayashi J, Sato E, Nomura M, et al. A case of paravertebral mediastinal chordoma without bone destruction. Skelet Radiol. 2012;41:1641–4.

    Article  Google Scholar 

  • Mehnert F, Beschorner R, Kuker W, Hahn U, Nagele T. Retroclival ecchordosis physaliphora: MR imaging and review of the literature. AJNR Am J Neuroradiol. 2004;25:1851–5.

    PubMed  Google Scholar 

  • Nishiguchi T, Mochizuki K, Tsujio T, Nishita T, Inoue Y. Lumbar vertebral chordoma arising from an intraosseous benign notochordal cell tumour: radiological findings and histopathological description with a good clinical outcome. Br J Radiol. 2010;83:e49–53.

    Article  CAS  PubMed  Google Scholar 

  • Nishiguchi T, Mochizuki K, Ohsawa M, et al. Differentiating benign notochordal cell tumors from chordomas: radiographic features on MRI, CT, and tomography. AJR Am J Roentgenol. 2011;196:644–50.

    Article  PubMed  Google Scholar 

  • Oner AY, Akpek S, Tali T, Ucar M. Giant vertebral notochordal rest: magnetic resonance and diffusion weighted imaging findings. Korean J Radiol. 2009;10:303–6.

    Article  PubMed Central  PubMed  Google Scholar 

  • Rotondo M, Natale M, Mirone G, Cirillo M, Conforti R, Scuotto A. A rare symptomatic presentation of ecchordosis physaliphora: neuroradiological and surgical management. J Neurol Neurosurg Psychiatry. 2007;78:647–9.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  • Shen J, Li CD, Yang HL, et al. Classic chordoma coexisting with benign notochordal cell rest demonstrating different immunohistological expression patterns of brachyury and galectin-3. J Clin Neurosci. 2011;18:96–9.

    Article  CAS  PubMed  Google Scholar 

  • Smith DS, French G, Miller M. Multifocal benign notochordal cell tumours. Histopathology. 2007;51:412–4.

    Article  CAS  PubMed  Google Scholar 

  • Souteyrand P, Bouvier C, Zink JV, et al. Benign notochordal cell tumour: case report. Diagn Interv Imaging. 2013;94:466–9.

    Article  CAS  PubMed  Google Scholar 

  • Terzi S, Mobarec S, Bandiera S, et al. Diagnosis and treatment of benign notochordal cell tumors of the spine: report of 3 cases and literature review. Spine (Phila Pa 1976). 2012;37:E1356–60.

    Article  Google Scholar 

  • Uglialoro AD, Beebe KS, Hameed M, Benevenia J. A rare case of intraosseous benign notochordal cell tumor of the coccyx. Orthopedics. 2009;32:445.

    Article  PubMed  Google Scholar 

  • Wang WL, Abramson JH, Ganguly A, Rosenberg AE. The surgical pathology of notochordal remnants in adult intervertebral disks: a report of 3 cases. Am J Surg Pathol. 2008;32:1123–9.

    Article  PubMed  Google Scholar 

  • Yamaguchi T, Yamato M, Saotome K. First histologically confirmed case of a classic chordoma arising in a precursor benign notochordal lesion: differential diagnosis of benign and malignant notochordal lesions. Skelet Radiol. 2002;31:413–8.

    Article  Google Scholar 

  • Yamaguchi T, Suzuki S, Ishiiwa H, Ueda Y. Intraosseous benign notochordal cell tumours: overlooked precursors of classic chordomas? Histopathology. 2004a;44:597–602.

    Article  CAS  PubMed  Google Scholar 

  • Yamaguchi T, Suzuki S, Ishiiwa H, Shimizu K, Ueda Y. Benign notochordal cell tumors: a comparative histological study of benign notochordal cell tumors, classic chordomas, and notochordal vestiges of fetal intervertebral discs. Am J Surg Pathol. 2004b;28:756–61.

    Article  PubMed  Google Scholar 

  • Yamaguchi T, Watanabe-Ishiiwa H, Suzuki S, Igarashi Y, Ueda Y. Incipient chordoma: a report of two cases of early-stage chordoma arising from benign notochordal cell tumors. Mod Pathol. 2005;18:1005–10.

    Article  PubMed  Google Scholar 

  • Yamaguchi T, Iwata J, Sugihara S, et al. Distinguishing benign notochordal cell tumors from vertebral chordoma. Skelet Radiol. 2008;37:291–9.

    Article  Google Scholar 

  • Yamamoto T, Yano S, Hide T, Kuratsu J. A case of ecchordosis physaliphora presenting with an abducens nerve palsy: a rare symptomatic case managed with endoscopic endonasal transsphenoidal surgery. Surg Neurol Int. 2013;4:13.

    Article  PubMed Central  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Yasuaki Nakashima MD, PhD .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer-Verlag London

About this chapter

Cite this chapter

Nakashima, Y. (2015). Benign Notochordal Cell Tumor. In: Santini-Araujo, E., Kalil, R., Bertoni, F., Park, YK. (eds) Tumors and Tumor-Like Lesions of Bone. Springer, London. https://doi.org/10.1007/978-1-4471-6578-1_38

Download citation

  • DOI: https://doi.org/10.1007/978-1-4471-6578-1_38

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-4471-6577-4

  • Online ISBN: 978-1-4471-6578-1

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics