Introduction: Leiomyosarcoma (LMS) is a rare malignant tumor arising from the smooth-muscle cells which rarely metastasizes to the bone and even less is found in the spine as a primary localization. In this article the authors describe a case of a peculiar localization of this neoplasm in the upper thoracic spine which required a tailored surgical treatment. Case Report: A 52-year-old female presented with an 8 weeks' history of gradually worsening myelopathy and sensory level in the upper thoracic spine. Pre-operation MRI scans showed an invasive mass neoplasm at T3 and T4 levels involving the vertebral bodies and pedicles with a significant cord compression. A 360° two-step approach was performed. The first operation consisted in a T1-T4 decompressive laminectomy with a sub-total tumor resection and pedicle fixation extending from C7 to T5. A second surgical step was performed through an anterior approach in order to improve the amount of tumor removal. Instrument fixation assured the stabilization of the spinal segment. Post-operative scans demonstrated an optimal decompression. Neurological symptoms gradually improved, and the patient benefitted from a gradual reversion of previous symptomatology and could go back to her previous life. Conclusion: LMS represents a challenging tumor due to an extremely aggressive behavior and a considerably high rate of recurrence which requires a tailored approach in terms of surgical strategy and follow-up. A exhaustive review of literature was performed in the attempt to rationalize a surgical strategy and correct management of this extremely rare neoplastic lesion.
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