Benign notochordal cell tumor

Benign notochordal cell tumors are vertebral lesions that are usually asymptomatic and discovered incidentally on imaging of the head or spine. As this is a poorly-recognised entity, it can often be confused with aggressive vertebral lesions, such as a chordoma, when it is seen on imaging.

Autopsy studies show benign notochordal cell tumors are extremely common with a reported incidence of up to 20% in cadaveric specimens 1. While only the larger of these lesions can be seen on imaging it is felt they are under-reported 2

The vast majority of lesions are asymptomatic but a small proportion of patients can present with chronic back pain and coccydynia 2.

The distribution is similar to chordomas:

  • clivus (50%)
  • vertebrae
  • sacrum/coccyx
  • vertebral body sclerosis
    • can extend to the cortex or involve the entire vertebra
  • preserved trabeculae
  • no cortical destruction
  • well-defined osseous lesions
    • T1: hypo- or isointense; may demonstrate hyperintense intra-lesional punctiform foci representing fat lobules due to entrapped bone marrow
    • T2: hyperintense
    • T1 C+ (Gd): usually no enhancement (in around 75% of cases)
    • DWI: no restricted diffusion
  • no soft tissue component

Often show no uptake on bone scintigraphy.

While there is a consensus that no specific treatment is required for asymptomatic lesions, the long-term malignant potential of benign notochordal cell tumors to transform into chordomas is not known and many advocate interval follow-ups 5.

Consider

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Article information

rID: 59319
Tag: cases
Synonyms or Alternate Spellings:
  • Benign notochordal cell tumour (BNCT)
  • Giant vertebral notochordal rest

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