Management and Outcome of Solitary Spinal Amyloidoma-A Systematic Literature Review

BACKGROUND: Solitary spinal amyloidoma (SSA) is a rare and poorly characterized disease. There are few cases described, and the knowledge of this neoplasm is limited. A more accurate description of demographics, clinical findings, and outcomes may be useful for a better understanding of this pathology, as well as therapeutic intervention, adding value to the research of localized amyloidosis. METHODS: A systematic search was carried out from when registries began until February 2020. We also include a case diagnosed and treated in our department. Descriptive statistics were used to evaluate data, demographics, clinical findings, diagnostic modalities, therapeutics, and finally neurologic outcomes. The Kaplan-Meier method was used to assess overall survival and progression-free survival. RESULTS: The final cohort comprises 35 patients. The mean age at diagnosis was 61.97 years, and 68.60% of the patients were male. SSA developed more frequently in the thoracic spine (48.60%), followed by the cervical spine (17.10%). Intradural lesions were rare, and the average neoplastic score for spinal instability was 9.5 points. The most common symptoms were impaired motor function (74.29%) and axial back pain (65.70%). After surgery, neurologic recovery was reported in 82.90% of cases. Mean progression-free survival and mean overall survival were 47.26 and 156.66 months. CONCLUSIONS: SSA is a rare subgroup of localized amyloidosis, usually being diagnosed in male patients between the sixth and eighth decades. The gold standard treatment seems to be surgical resection. SSA patients have excellent long-term survival and a low rate of local recurrence.