PubMed · 13167391
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A CIRLA. 1953. [Chordomas].. https://pubmed.ncbi.nlm.nih.gov/13167391/
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BACKGROUND: Following recent progress in radiological and surgical techniques for treating skull base tumours, more attention should be paid to the relatively rare phenomenon of intradural spinal seeding in cases of clival chordoma. CASE PRESENTATION: A 53-year-old man harboured intradural spinal seeding from a clival chordoma 11 years after his initial surgical treatment. He had undergone skull base surgery five times with extra- and intradural approaches, and adjuvant radiotherapy. His complaints of lumbago, paraparesis, and sensory disturbance of the lower left extremity prompted us to carry out imaging studies of the spinal cord, which revealed multiple intradural, extramedullary mass lesions at the thoracic and lumbosacral spinal level. The tumours were histopathologically diagnosed as spinal seeding of the clival chordoma with high proliferative potential (Ki-67 labelling index of 8.4%). INTERPRETATION: As long-term local control of skull base chordoma appears to be improving by the introduction of skull base surgical techniques and adjuvant radiotherapy, spinal intradural seeding might emerge as a potential complication. Close observation and timely imaging studies for spinal seeding of skull base chordoma would be required in long-term postoperative follow-ups, particularly for those patients with highly proliferative tumours.
Some chordomas have a very poor prognosis because of their aggressive growth nature, but the efficacy of repeat operations for these cases has not been well documented. This report concerns 3 patients with aggressive chordoma of the clivus, who underwent operations 6 to 12 times over a period of 8 to 17 years because of symptomatic regrowth. Overall mean interval between repeat operations was 18 months with a range from 5 to 57 months and survival times were 9 to19 years after the first surgery. Main symptoms before each operation were diplopia and visual disturbance. Repeat palliative operations by intentional extradural debulking of the tumour to decompress offending neural structures, as well as maximal removal of the tumour, using appropriate skull base approaches, can mitigate progressive symptoms, and may result in better quality and some prolongation of life, although our patients gradually deteriorated neurologically throughout the clinical course.