Distinguishing benign notochordal cell tumors from vertebral chordoma

Abstract

Objective The objective was to characterize imaging findings of benign notochordal cell tumors (BNCTs). Design and patients Clinical and imaging data for 9 benign notochordal cell tumors in 7 patients were reviewed retrospectively. Conventional radiographs (n=9), bone scintigrams (n=2), computed tomographic images (n=7), and magnetic resonance images (n=8) were reviewed. Eight of the 9 lesions were stained with hematoxylin-eosin and microscopically examined. Results There were 3 male and 4 female patients with an age range of 22 to 55 years (average age, 44 years). Two patients had two lesions at different sites. The lesions involved the cervical spine in 4 patients, the lumbar spine in 2, the sacrum in 2, and the coccyx in 1. The most common symptom was mild pain. The lesions of 2 patients were found incidentally during imaging studies for unrelated conditions. Five patients underwent surgical procedures. One patient died of surgical complications. All other patients have been well without recurrent or progressive disease for 13 to 84 months. Radiographs usually did not reveal significant abnormality. Five lesions exhibited subtle sclerosis and 1 showed intense sclerosis. Technetium bone scan did not reveal any abnormal uptake. Computed tomography images had increased density within the vertebral bodies. The lesions had a homogeneous low signal intensity on T1-weighted magnetic resonance images and a high intensity on T2-weighted images without soft-tissue mass. Microscopically, lesions contained sheets of adipocyte-like vacuolated chordoid cells without a myxoid matrix. Conclusions Benign notochordal cell tumors may be found during routine clinical examinations and do not require surgical management unless they show extraosseous disease. These tumors should be recognized by radiologists, pathologists, and orthopedic surgeons to prevent operations, which usually are extensive.

Case Report: An Unusual Osseous Lesion

“…Benign notochordal cell tumors are intraosseous lesions which can occur along the midline of the craniospinal axis, particularly in the saccrococcygeal or sphenooccipital region [8,10]. A recent autopsy series found BNCTs in 20% of 100 cadavers, with a mean age of 63 years (7 to 82 years), with a distribution of 12% in saccrococcygeal vertebrae, 11.5% in the clival region, 5% in cervical vertebrae, 2% in lumbar vertebrae and none in the thoracic vertebrae [8].…”

“…As such, terms like "benign chordoma" or "giant notochordal hamartoma of intraosseous origin" have been suggested [1]. Finally, the term intraosseous benign notochordal cell tumor (BNCT) was recently adopted, including both small lesions found incidentally and larger lesions that have become detectable on radiological images or have been symptomatic [1,9,10].…”

“…Benign notochordal cell tumors are usually asymptomatic [10]. When they do give symptoms, the most prominent is back pain [1,3].…”

Metastatic Chordoma: Report of the Two Cases and Review of the Literature

“…Chordomas have a predilection for the craniospinal axis and are thought to arise from notochordal rests which evolve into nucleus pulposus, explaining the predominant midline and paramedian locations [6]. Newer literature challenges this traditional concept suggesting that chordomas arise from pre-existing benign notochordal cell tumours [7][8][9][10]. Chordomas present between the fourth and seventh decade with a male to female ratio of 2:1 [6,11].…”

Benign notochordal cell tumor: case report

“…Asemptomatik hastalarda uzun süreli takip ve seri görüntüleme ile izlem gerekmektedir. Ağrıya neden olan BnHT› ler için konservatif tedavi semptomların iyileştirilmesinde başarılı olmakla birlikte, eksizyon ile tam iyileşme olabilir (10,11). Bunun aksine, spinal / sakral kordomalar, önemli morbiditeye neden olabilen, en-blok cerrahi rezeksiyon ile tedavi edilir (1).…”