Presentation, Management, and Outcome of Primary Leiomyosarcoma of the Spine: A Systematic Review.

OBJECTIVE: Primary spinal leiomyosarcoma (PSL) is extremely rare. A case is presented, followed by a systematic review establishing the consensus on presentation, diagnosis, management, and outcomes. Comparison is made with metastatic spinal leiomyosarcoma (MSL). METHODS: A systematic review was conducted in line with the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. Eligibility criteria were decided before the literature search was conducted. Data were extracted and analyzed. RESULTS: A total of 397 articles were identified, 25 of which conformed to the eligibility criteria. Thirty-four cases were included in the analysis. PSL had a female preponderance (69.2%), with back pain being the most common presenting symptom (60.9%). Neurologic signs were present in most (69.6%), with tumors typically in the thoracic spine (46.9%). Diagnosis was primarily made using magnetic resonance imaging (64.7%) and computed tomography (55.9%), with a histologic sample being obtained in all cases. Most patients underwent operative management (91.2%), with variable use of neoadjuvant and adjuvant therapies. Operative approach differed greatly and outcome after surgical management was stated in 48.4% of patients, all noting an improvement from presentation. Patient follow-up was limited (median, 7 months), with most patients being free of disease (43.8%). CONCLUSIONS: PSL diagnosis is challenging, with positron emission tomography-computed tomography and histopathologic sampling playing an important role. There is a limited evidence base for the treatment strategies used but surgical management is key, with generally good outcomes. Prognosis for PSL seems to be better than for MSL. There is scope for more dedicated research in PSL and MSL.