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. 2008 Apr;37(4):291-9.
doi: 10.1007/s00256-007-0435-y. Epub 2008 Jan 8.

Distinguishing benign notochordal cell tumors from vertebral chordoma

Takehiko Yamaguchi  1 , Jun Iwata, Shinsuke Sugihara, Edward F McCarthy Jr, Michiaki Karita, Hideki Murakami, Norio Kawahara, Hiroyuki Tsuchiya, Katsuro Tomita
Affiliations

Distinguishing benign notochordal cell tumors from vertebral chordoma

Takehiko Yamaguchi et al. Skeletal Radiol. 2008 Apr.

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.

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Figures

Fig. 1
Fig. 1
A 30-year-old man who complained of low back pain (patient 1). a Lateral radiograph of the lumbar spine reveals vague sclerosis in the fourth lumbar vertebra. b Axial computed tomography (CT) scan of the fourth lumbar vertebra demonstrates significant sclerosis in the center of the body, partly extending to the cortex. c Sagittal T1-weighted spin echo magnetic resonance (MR) image reveals a large intraosseous lesion with low signal intensity. The normal bone marrow signal is preserved in the anterior and posterior portions of the body. d Sagittal T2-weighted MR image shows slightly bright signal intensity intermingled with intermediate signal. e Sagittal gadolinium-DTPA-enhanced MR image does not demonstrate any enhancement. No soft tissue mass is recognized
Fig. 2
Fig. 2
A 53-year-old woman who was examined for ossification of the posterior longitudinal ligament at the sixth to seventh cervical vertebrae (patient 2). A lesion was found incidentally. a Lateral radiograph reveals faint or vague osteosclerosis in the 6th cervical vertebral body. The physiological lordosis of the cervical spine is decreased. b Axial CT scan reveals diffuse sclerosis within the vertebral body. c Sagittal T1-weighted spin echo MR image reveals low signal intensity in almost the entire marrow space of the sixth cervical vertebra. d Sagittal T2-weighted MR image reveals intensely high signal replacing the entire marrow space. No soft tissue mass is evident
Fig. 3
Fig. 3
A 43-year-old woman who was found to have two separate abnormalities in the lower spine during an imaging study for a traffic accident (patient 7). a Lateral radiograph of the lumbar spine and sacrum reveals intense sclerosis of the entire fifth lumbar vertebral body and mild sclerosis in the cephalad portion of the sacrum. b Sagittal T1-weighted MR image reveals homogeneous low signal intensity in both the fifth lumbar and second sacral vertebrae
Fig. 4
Fig. 4
A 52-year-old man who complained of mild upper back pain (patient 5). a Sagittal T2-weighted MR image reveals an intraosseous lesion with high signal intensity in the fifth cervical vertebra at initial presentation. b Sagittal T2-weighted MR image demonstrates no progressive disease 14 months after needle biopsy. No extraosseous tumor extension or enlargement is recognized
Fig. 5
Fig. 5
Photomicrographs of benign notochordal cell tumors. a Low power magnification (patient 1) reveals solid sheets of adipocyte-like vacuolated cells. The affected bone trabeculae are sclerotic. Some islands of non-neoplastic hematopoietic bone marrow are seen in the lesion (hematoxylin-eosin [HE] stain). b Higher power magnification (patient 1) reveals a solid sheet of vacuolated tumor cells of various sizes. The nuclei are oval and appear bland (HE stain). c Some tumor cells (patient 1) are multivacuolated and centrally located nuclei are mildly polymorphic. They are reminiscent of lipoblasts (HE stain). d Biopsy specimen (patient 5) reveals sheets of vacuolated cells with pyknotic nuclei between bone trabeculae. The tumor cells may be mistaken for degenerative fatty marrow (HE stain). e The tumor cells (patient 5) stain immunohistochemically positive for cytokeratin (AE1/AE3) indicating notochordal cell origin (SAB immunohistochemical stain)
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References

    1. {'text': '', 'ref_index': 1, 'ids': [{'type': 'DOI', 'value': '10.1007/s002560050528', 'is_inner': False, 'url': 'https://doi.org/10.1007/s002560050528'}, {'type': 'PubMed', 'value': '10450882', 'is_inner': True, 'url': 'https://pubmed.ncbi.nlm.nih.gov/10450882/'}]}
    2. Darby AJ, Cassar-Pullicino VN, McCall IW, Jaffray DC. Vertebral intra-osseous chordoma or giant notochordal rest? Skeletal Radiol 1999; 28: 342–346. - PubMed
    1. {'text': '', 'ref_index': 1, 'ids': [{'type': 'DOI', 'value': '10.1007/s002560100422', 'is_inner': False, 'url': 'https://doi.org/10.1007/s002560100422'}, {'type': 'PubMed', 'value': '11810168', 'is_inner': True, 'url': 'https://pubmed.ncbi.nlm.nih.gov/11810168/'}]}
    2. Mirra JM, Brien EW. Giant notochordal hamartoma of intraosseous origin: a newly reported benign entity to be distinguished from chordoma. Report of two cases. Skeletal Radiol 2001; 30: 698–709. - PubMed
    1. {'text': '', 'ref_index': 1, 'ids': [{'type': 'DOI', 'value': '10.1007/s00256-002-0514-z', 'is_inner': False, 'url': 'https://doi.org/10.1007/s00256-002-0514-z'}, {'type': 'PubMed', 'value': '12107574', 'is_inner': True, 'url': 'https://pubmed.ncbi.nlm.nih.gov/12107574/'}]}
    2. Yamaguchi T, Yamato M, Saotome K. First histologically confirmed case of a classic chordoma arising in a precursor benign notochordal lesion: differential diagnosis of benign and malignant notochordal lesions. Skeletal Radiol 2002; 31: 413–418. - PubMed
    1. {'text': '', 'ref_index': 1, 'ids': [{'type': 'DOI', 'value': '10.1111/j.1365-2559.2004.01877.x', 'is_inner': False, 'url': 'https://doi.org/10.1111/j.1365-2559.2004.01877.x'}, {'type': 'PubMed', 'value': '15186275', 'is_inner': True, 'url': 'https://pubmed.ncbi.nlm.nih.gov/15186275/'}]}
    2. Yamaguchi T, Suzuki S, Ishiiwa H, Ueda Y. Intraosseous benign notochordal cell tumors: overlooked precursors of classic chordomas? Histopathology 2004; 44: 597–602. - PubMed
    1. {'text': '', 'ref_index': 1, 'ids': [{'type': 'DOI', 'value': '10.1097/01.pas.0000126058.18669.5d', 'is_inner': False, 'url': 'https://doi.org/10.1097/01.pas.0000126058.18669.5d'}, {'type': 'PubMed', 'value': '15166667', 'is_inner': True, 'url': 'https://pubmed.ncbi.nlm.nih.gov/15166667/'}]}
    2. Yamaguchi T, Suzuki S, Ishiiwa H, Shimizu K, Ueda Y. 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 2004; 28: 756–761. - PubMed