Lumbar vertebral chordoma arising from an intraosseous benign notochordal cell tumour: radiological findings and histopathological description with a good clinical outcome

Abstract

Benign notochordal cell tumours have recently been described as intraosseous benign lesions of notochordal cell origin. The lesions are found in vertebral bodies in 20% of autopsy studies and are a potential precursor of chordoma. We report a rare case of lumbar vertebral chordoma that was thought to arise from a benign intraosseous notochordal cell tumour and which showed significant osteosclerotic change. Radiologically, the lumbar vertebral mass lesion showed hyperintensity on T2 weighted images, with scanty enhancement on post-contrast T1 weighted MR images. High uptake corresponding to the mass was noted on fluorine-18-fluorodeoxyglucose positron emission tomography. Bone biopsy revealed proliferation of the physaliphorous cells between thickened bone trabeculae; no nuclear mitosis was observed. Although the mass was diagnosed clinically as spinal chordoma, histopathology contained both benign notochordal cell tumour and conventional chordoma. After heavy particle (11C)-charged radiation therapy was applied to the lesion with a sufficient radiation field margin, the tumour volume significantly decreased and there was improvement in the patient's symptoms. On follow-up radiological studies, the tumour had markedly regressed and there was no tumour regrowth or distant metastasis. In this case report, benign notochordal cell tumour and conventional chordoma are histopathologically identified in the L1 vertebral body, which contains osteosclerotic and osteolytic areas. It is suggested that the benign notochordal cell tumour coexists with a conventional chordoma and that this histopathological finding supports a hypothetical relationship between benign notochordal cell tumour and chordoma.