abstract
- PURPOSE: The objective of this study was to examine the long-term outcomes of pediatric patients who underwent surgical resection for lipoblastoma and lipoblastomatosis (LB/LBM). METHODS: A single-center retrospective study of pediatric patients with LB/LBMs seen between 1991 and 2015 was conducted. A systematic review, including studies published prior to late August 2018, was performed. Using a random effect meta-analysis, pooled weighted proportions and unadjusted odds ratios (OR) with 95% confidence intervals (CI) were calculated. RESULTS: The retrospective study included 16 patients, while the systematic review included 19 published studies consisting of 381 patients. Among 329 (82%) patients with follow-up information, the pooled recurrence rate was 16.8% (95% CI 10.9-23.5%; I2 = 59%). The reported time to recurrence ranged from < 1 to 8 years. Recurrence risk was greater for incomplete (n = 34) than complete resection (n = 150): OR 11.4 (95% CI 3.0-43.6; I2 = 43%). LBMs (n = 35) had a greater recurrence risk than LBs (n = 116): OR 5.5 (95% CI 1.9-15.9; I2 = 0%). Recurrences were higher for studies with approximately ≥ 3 years of follow-up versus studies with < 3 years of follow-up. CONCLUSION: Recurrences are more likely to occur with LBMs and/or incomplete resection. Follow-up beyond 3-5 years should be considered given that the recurrence risk appears to be greater in the long-term.