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Imaging of spinal chordoma and benign notochordal cell tumor (BNCT) with radiologic pathologic correlation

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Abstract

Benign notochordal cell tumor (BNCT) and chordoma are neoplasms of notochordal differentiation. BNCT represents notochordal rests, commonly an incidental lesion present in the spine in 19% of cadaveric specimens. BNCTs are often radiographically occult. CT of BNCT frequently reveals patchy sclerosis between areas of maintained underlying trabeculae. BNCT demonstrates marrow replacement on T1-weighted MR images with high signal intensity on T2-weighting. BNCTs are frequently smaller than 35 mm and lack significant enhancement, bone destruction, cortical permeation, or soft tissue components. Biopsy or surgical resection of BNCT is usually not warranted, although imaging surveillance may be indicated. Chordoma is a rare low-grade locally aggressive malignancy representing 1–4% of primary malignant bone tumors. Chordoma is most frequent between the ages of 50–60 years with a male predilection. Clinical symptoms, while nonspecific and location dependent, include back pain, numbness, myelopathy, and bowel/bladder incontinence. Unfortunately, lesions are often large at presentation owing to diagnosis delay. Imaging of chordoma shows variable mixtures of bone destruction and sclerosis, calcification (50–70% at CT) and large soft tissue components. MR imaging of chordoma reveals multilobulated areas of marrow replacement on T1-weighting and high signal intensity on T2-weighting reflecting the myxoid component within the lesion and areas of hemorrhage seen histologically. Treatment of chordoma is primarily surgical with prognosis related to resection extent. Unfortunately, complete resection is often not possible (21–75%) resulting in high local recurrence incidence (19–75%) and a 5-year survival rate of 45–86%. This article reviews and illustrates the clinical characteristics, pathologic features, imaging appearance spectrum, treatment, and prognosis of BNCT and spinal chordoma.

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Acknowledgements

The authors gratefully acknowledge the support of Tracee Lee Marshall, Evan B. Greenfield, and former fellows Andrew J. Degnan, MD, and Augustus Suhardja, MD, for manuscript preparation, and without whom this project would not have been possible. We also thank all attendees of the AIRP radiologic pathology courses (past, present, and future) for their contribution to our series of cases.

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  1. Musculoskeletal Imaging and Neuroradiology, ACR Institute for Radiologic Pathology (AIRP), 1100 Wayne Avenue, Suite 1020, Silver Spring, MD, 20910, USA

    Mark D. Murphey, Matthew J. Minn, Kelly K. Koeller & Robert Y. Shih

  2. Uniformed Services University of the Health Sciences, 4301 Jones Bridge Road, Bethesda, MD, 20814, USA

    Mark D. Murphey & Robert Y. Shih

  3. Department of Radiology, Walter Reed National Military Medical Center, 8901 Wisconsin Ave., Bethesda, MD, 20889, USA

    Mark D. Murphey, Matthew J. Minn & Robert Y. Shih

  4. Department of Radiology, Scripps Green Hospital, 10666 N. Torrey Pines Road, La Jolla, CA, 92037, USA

    Matthew J. Minn

  5. Joint Pathology Center, 606 Stephen Sitter Avenue, Silver Spring, MD, 20910-1290, USA

    Alejandro Luiña Contreras

  6. Department of Radiology, Head and Neck Cancer Center, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA

    Kelly K. Koeller

  7. Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA

    Carrie Y. Inwards

  8. Department of Pathology, Nikko Medical Center, Dokkyo Medical University, 632 Takatoku, Nikko, Tochigi, 321-2593, Japan

    Takehiko Yamaguchi

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Murphey, M.D., Minn, M.J., Contreras, A.L. et al. Imaging of spinal chordoma and benign notochordal cell tumor (BNCT) with radiologic pathologic correlation. Skeletal Radiol 52, 349–363 (2023). https://doi.org/10.1007/s00256-022-04158-7

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