Imaging of notochordal tumors

Prabhakar Rajiah, Hakan Ilaslan, Murali Sundaram

Research output: Chapter in Book/Report/Conference proceedingChapter


Notochord is a midline axial structure that is a major structural organizer in the development of the spine. Tumors originating from notochordal remnants are rare and are typically seen in the midline of the body, commonly in the sacrococcygeal and spheno-occipital regions. Classic chordoma is a malignant notochordal tumor that is pathologically characterized by strands of atypical chordoid cells and myxoid matrix. Radiologically, chordoma presents as an osteolytic mass, with a large calcified soft tissue mass that shows contrast enhancement. Chordomas are locally aggressive and are treated with radical resection and adjuvant radiation therapy. Chondroid chordoma and extra-axial chordoma are variants of chordoma that are associated with a better prognosis than that of classic chordoma. Dedifferentiated chordoma is a histogenetically distinct high-grade tumor arising in a pre-existing chordoma;prognosis in these cases depends on the dedifferentiated element, which is usually a high-grade spindle cell tumor. Benign notochordal cell tumors (BNCT) are intra-osseous benign lesions of notochordal cell origin, with clinical and histopathological features distinct from those of chordoma and notochordal rests. Pathologically, there is no myxoid matrix with BNCT as there is with chordoma. Radiographs and scintigraphy results are usually normal with BNCT, whereas computed tomography shows subtle sclerosis with no evidence of osteolysis and magnetic resonance imaging shows homogeneous low T1 and high T2 signal without contrast enhancement or soft tissue mass. With long-term follow-up, these benign tumors do not show progression to chordoma. Incipient chordoma has histological features between those of BNCT and chordoma. For all tumors of notochordal origin, pathological features should be correlated with imaging findings to ensure accurate diagnosis and appropriate management.

Original languageEnglish (US)
Title of host publicationBone Tumors
Subtitle of host publicationSymptoms, Diagnosis and Treatment
PublisherNova Science Publishers, Inc.
Number of pages10
ISBN (Print)9781626181908
StatePublished - May 1 2013

ASJC Scopus subject areas

  • General Biochemistry, Genetics and Molecular Biology
  • General Medicine


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