Skip to main content

Notochordal Tumours

  • Chapter

Part of the book series: Medical Radiology ((Med Radiol Diagn Imaging))

Key Points

• Notochordal tumors may be benign or malignant

• Notochordal tumors have a predilection for the spheno-occipital and sacral regions

• Benign notochordal tumors tend to be detected on MRI but demonstrate little or no abnormality on radiographs, CT or bone scintigraphy.

• Chordoma accounts for 50% of malignant sacral tumors.

• Sacral chordomas are predominently osteolytic with 90% showing varying degrees of calcification on CT, all showing an anterior soft tissue mass and 77% a posterior soft tissue mass on MRI.

• Biopsy identification of choirdoid tissue needs to be carefully correlated with MRI and CT appearances to decide if it represents BNCT or chordoma.

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

Buying options

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   149.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   199.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD   219.99
Price excludes VAT (USA)
  • Durable hardcover 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

Learn about institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  • Chauvel A, Taillat F, Gille O, et al. (2005) Giant vertebral notochordal rest: a new entity distinct from chordoma. Histopathology 47:643–652

    Article  Google Scholar 

  • Cheung EY, Ozerdemogle RA, Transfeldt EE, Thompson RC (1999) Lumbosacral chordoma: prognostic factors and treatment. Spine 24:1639–1645

    Article  Google Scholar 

  • Congdon CC (1952) Benign and malignant chordomas a clinico-anatomical study of twenty-two cases. Am J Surg Pathol 28:793–821

    CAS  Google Scholar 

  • Corsi A, De Maio F, Mancini F, Ippolito E, Riminucci M, Bianco P (2008) Notochordal inclusions in the vertebral bone marrow. J Bone Miner Res 23:572–575

    Article  PubMed  Google Scholar 

  • Crapanzano JP, Ali SZ, Ginsberg MS, Zakowski MF (2001) Chordoma; cytologic study with histologic and radiologic correlation. Cancer 93:40–51

    Article  PubMed  CAS  Google Scholar 

  • Darby AJ, Cassar-Pullicino VN, McCall IW, Jaffray DC (1999) Vertebral intra-osseous chordoma or giant notochordal rest? Skeletal Radiol 28:342–346

    Article  PubMed  CAS  Google Scholar 

  • De Bruine FT, Kroon HM (1988) Spinal chordoma: radiological features in 14 cases. AJR Am J Roentgenol 150:861–863

    PubMed  Google Scholar 

  • Deshpande V, Nielsen GP, Rosenthal DI, Rosenberg AE (2007) Intra-osseous benign notochord cell tumors (BNCT): further evidence supporting a relationship to chordoma. Am J Surg Pathol 31:1573–1577

    Article  PubMed  Google Scholar 

  • Di Girolamo S, Ottoviani F, Floris R, Bruno E, Napolitano B, Schillaci O (2005) Indium III Pentetreotide single photon emission computed tomography (In III pentetreotide Spect) a new technique to evaluate sematostatin receptors in chordomas. J Laryngol OTOL 119:405–408

    PubMed  Google Scholar 

  • Firooznia H, Pinto RS, Baruch HH, Zausner J (1976) Chordoma: radiographic evaluation of 20 cases. Am J Roentgenol 127:797–805

    CAS  Google Scholar 

  • Fletcher CDM, Unni KK, Mertens F (eds) (2002) WHO classification: Pathology and genetics of tumours of soft tissue and bone. IARC Press, Lyon

    Google Scholar 

  • Fuchs B, Dickey ID, Yaczemski MJ, Inwards CY, Sims FH (2005) Operative management of sacral chordoma. J Bone Joint Surg Am 87:2211–2216

    Article  PubMed  Google Scholar 

  • Hanna SA, Aston WJ, Briggs TW, Cannon SR, Saifuddin A (2008) Sacral chordoma: can local recurrence after sacrectomy be predicted? Clin Orthop Relat Res 466:2217–2223

    Article  PubMed  CAS  Google Scholar 

  • Heffelfinger MJ, Dahlin DC, MacCarty CS, Beabout JW (1973) Chordomas and cartilaginous tumours at the skull base. Cancer 32:410–420

    Article  PubMed  CAS  Google Scholar 

  • Howitz T (1941) Chordal ectopia and its possible relation to chordoma. Arch Path 31:354–362

    Google Scholar 

  • Hudson TM, Galceran M (1983) Radiology of sacrococcygeal chordoma. Difficulties in detecting soft tissue extension. Clin Orthop Relat Res 175:237–242

    PubMed  Google Scholar 

  • Kyriakos M, Totty WG, Lenke LG (2003) Giant vertebral notochordal rest: a lesion distinct from chordoma: discussion of an evolving concept. Am J Surg Pathol 27:396–406

    Article  PubMed  Google Scholar 

  • Luschka H (1856) Die Altersveränderungen der Zwischenwirbelknorpel. Virchows Arch Path Anat 9:311–327

    Google Scholar 

  • Mehnert F, Beschorner R, Kuker W, Hahn U, Nagele T (2004) Retroclival ecchordosis physalphora: MR imaging and review of the literature. AJNR 25:1851–1855

    PubMed  Google Scholar 

  • Meis JM, Raymond AK, Evans HL, Charles RE, Giraldo AA (1987) “Dedifferentiated” chordoma. A clinico-pathologic and immunohistochemical study of three cases. Am J Surg Path 11:516–525

    Article  PubMed  CAS  Google Scholar 

  • Meyer JE, Lepke RA, Undfors KK, Pagani JJ, Hirschy JC, Hayman LA, Momose KG, McGinnis B (1984) Chordomas: their CT appearances in the cervical thoracic and lumbar spine. Radiology 153:693–696

    PubMed  CAS  Google Scholar 

  • Mirra JM, Brien EW (2001) Giant notochordal hamartoma of intra-osseous origin: a newly reported benign entity to be distinguished from chordoma. Report of two cases. Skeletal Radiol 30:698–709

    Article  PubMed  CAS  Google Scholar 

  • Müller H (1858) Über das Vorkommen von Resten der chorda dorsalis bei Menschen nach der Geburt und über ihr Verhältniss zu den Gallertgeschwulsten am Clivus Ztschr f. rat Med 2:202–229

    Google Scholar 

  • Murakami H, Kawahara N, Gabata T, Nambu K, Tomita K (2003) Vertebral body osteonecrosis without vertebral collapse. Spine 28:E323–E328

    Article  PubMed  Google Scholar 

  • Murakami H, Kawahara N, Tomita K, Yamaguchi T Re, Murakami H, Kawahara N, Gabata T et al. (2005) Vertebral body osteonecrosis without vertebral collapse (letter to the editor). Spine 30:593–594

    Article  PubMed  Google Scholar 

  • Murphey MD, Andrews CL, Flemming DJ et al. (1996) Primary tumors of the spine: radiologic-pathologic correlation. Radiographics 16:1131–1158

    PubMed  CAS  Google Scholar 

  • Ng SH, Ko SK, Wan YL, Tang LM, Ho YS (1988) Cervical ecchordosis physaliphora: CT and MR features. BJR 171:329–331

    Google Scholar 

  • Oner AY, Akpek S, Tali T, Ucar M (2009) Giant vertebral notochordal rest: magnetic resonance and diffusion weighted imaging. Korean J Radiol 10:303–306

    Article  PubMed  Google Scholar 

  • Pazzaglia UE, Salisbury JR, Byers PD (1989) Development and involution of the notochord in the human spine. JRSM 82:413–415

    CAS  Google Scholar 

  • Radl R, Leithner A, Kastner N, Bodo K, Nader A, Windhager R (2005) Disappearance of a cervical spine chordoma after non-operative treatment. A case report. J Bone Joint Surg Am 87:1822–1827

    Article  PubMed  Google Scholar 

  • Ribbert H (1894) Ueber die Ecchondrosis physalifora spenooccipitalis. Zentralbl. F. allg. Path u path. Anat 5:457–461

    Google Scholar 

  • Rosenberg AE, Brown GA, Bhan AK, Lee JM (1994) Chondroid chordoma – a variant of chordoma. A morphologic and immunohisto chemical study. Am J Clin Pathol 101:36–41

    PubMed  CAS  Google Scholar 

  • Rosenthal DI, Scott JA, Mankin HJ, Wismer GL, Brady TJ (1985) Sacrococcygeal chordoma: magnetic resonance imaging and computed tomography. AJR Am J Roentgenol 145:143–147

    PubMed  CAS  Google Scholar 

  • Rossleigh MA, Smith J, Yeh SD (1986) Scintigraphic features of primary sacral tumours. J Nuc Med 27:627–630

    CAS  Google Scholar 

  • Salisbury JR (1993) The pathology of the human notochord. J Pathol 171:253–255

    Article  PubMed  CAS  Google Scholar 

  • Salisbury JR, Deverell MH, Cookson MJ, Whimster WF (1993) Three-dimensional reconstruction of human embryonic notochords: clue to the pathogenesis of chordoma. J Pathology 171:59–62

    Article  CAS  Google Scholar 

  • Sciubba D, Chi J, Rhines L, Gokasian Z (2008) Chordoma of the spinal column. Neurosurg Clin North Am 19:5–15

    Google Scholar 

  • Smolders D, Wang X, Drevelengas A, Vanhoenacker F, De Schepper AM (2003) Value of MRI in the diagnosis of non-clival, non-sacral chordoma. Skeletal Radiol 32:343–350

    PubMed  CAS  Google Scholar 

  • Steenberghs J, Kiekens C, Menten J, Monstrey J (2002) Intradural chordoma without bone involvement case report and review of the literature. J Neurosurg 97:94–97

    PubMed  Google Scholar 

  • Stewart MJ, Burrow J le F (1923) Ecchordosis physaliphora spheno-occipitalis. J Neurol Psychopath 4:218–220

    Article  Google Scholar 

  • Sung MS, Lee GK, Kang HS, Kwon ST, Park JG, Suh JS, Cho GH, Lee SM, Chung MH, Resnick D (2005) Sacrococcygeal chordoma: MR imaging in 30 patients. Skeletal Radiol 34:87–94

    Article  PubMed  Google Scholar 

  • Tirabosco R, Mangham DC, Rosenberg AE, Vujovic S, Bousdras K, Pizzolitto S, De Maglio G, den Bakker MA, Di Francesco L, Kalil RK, Athanasou NA, O’Donnell P, McCarthy EF, Flanagan AM (2008) Brachyury expression in extra-axial skeletal and soft tissue chordomas: a marker that distinguishes chordoma from mixed tumor/myoepithelioma/parachordoma in soft tissue. Am J Surg Pathol 32(4):572–580

    Article  PubMed  Google Scholar 

  • Ulich TR, Mirra JN (1982) Echordosis physaliphora vertebralis. Clin Orthop 163:282–289

    PubMed  Google Scholar 

  • Virchow R (1847) Untersuchungen über die Entwicklung des Schädelgrundes im gesunden und krankhaften Zustande und über den Einfluss derselben auf Schädelform, Gesichtsbildung und Gehirnbau. G Reimer, Berlin, p 47

    Google Scholar 

  • Vujovic S, Henderson S, Presneau N, Odell E, Jacques TS, Tirabosco R, Boshoff C, Flanagan AM (2006) Brachyury, a crucial regulator of notochordal development, is a novel biomarker for chordomas. J Pathol 209(2):157–165

    Article  PubMed  CAS  Google Scholar 

  • Wang AM, Joachim CL, Shillito J Jr, Morris JH, Zanani AA, Rumbaugh CL (1984) Cervical chordoma presenting with intervertebral foramen enlargement mimicking neurofibroma findings. J Comput Assist Tomogr 8:529–532

    Article  PubMed  CAS  Google Scholar 

  • Wippold FJ, Koeller KK, Smirniotpoulos JG (1999) Clinical and imaging features of cervical chordoma. AJR AM J Roentgenol 172:1423–1426

    PubMed  Google Scholar 

  • Yamaguchi T, Yamato M, Saotome K (2002) First histologically confirmed case of a classic chordoma arising in a precursor benign botochodral lesion; differential diagnosis of benign and malignant notochordal lesions. Skeletal Radiol 31:413–418

    Article  PubMed  Google Scholar 

  • Yagamuchi T, Suzuki S, Ishiiwa H, Shimizu K, Ueda Y (2004a) Benign notochordal cell tumors: a comparative histological study of benign notochordal cell tumors, classic chordomas and notochordal vestiges of fetal intervertebral disks. Am J Surg Pathol 28:756–761

    Article  Google Scholar 

  • Yamaguchi T, Suzuki S, Ishiiwa H, Ueda Y (2004b) Intraosseous benign notochordal cell tumors: overlooked precursors of classic chordomas? Histopathology 44:597–602

    Article  CAS  Google Scholar 

  • Yagamuchi T, Wannabe-Ishiiwa H, Suzuki S, Igarashi Y, Ueda Y (2005) Incipient chordoma: a report of two cases of early-stage chordoma. Mod Pathol 18:1005–1010

    Article  Google Scholar 

  • Yamaguchi T, Iwata J, Sugihara S, McCarthy EF Jr, Karita M, Murakami H, Kawahara N, Tsuchiya H, Tomita K (2008) Distinguishing benign notochordal cell tumours from vertebral chordoma. Skeletal Radiol 37:291–300

    Article  PubMed  Google Scholar 

  • York JE, Kaczaraj A, Abi-Said D, Fuller GN, Skibber JM, Janjan NA, Gokaslan ZL (1999) Sacral chordoma: 40-year experience at a major cancer center. Neurosurgery 44:74–79

    Article  PubMed  CAS  Google Scholar 

  • Zhang H, Yoshikawa K, Tamura K, Sagou K, Tian M, Suhara T, Kandatsu S, Suzuki K, Tanada S, Tsujii H (2004) Carbon 11-methionine emission tomography imaging of chordoma. Skeletal Radiol 33(9):524–530

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2009 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Cassar-Pullicino, V., Mangham, D. (2009). Notochordal Tumours. In: Davies, A., Sundaram, M., James, S. (eds) Imaging of Bone Tumors and Tumor-Like Lesions. Medical Radiology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-77984-1_20

Download citation

  • DOI: https://doi.org/10.1007/978-3-540-77984-1_20

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-77982-7

  • Online ISBN: 978-3-540-77984-1

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics