Risk Factors for Recurrence and the Role of Radiotherapy in Low-grade Myofibroblastic Sarcoma: A Systematic Review

Low-grade myofibroblastic sarcoma (LGMS) is a rare malignancy that commonly occurs in the head and neck region. The role of radiotherapy has been unclear in treating LGMS and the risk factors for recurrence have remained undefined. The objective of this study is to determine risk factors for the recurrence of LGMS in the head and neck as well as the role of radiotherapy in the treatment of LGMS. A comprehensive review of the literature was performed through Pubmed leading to the inclusion of 36 articles after our inclusion and exclusion criteria were applied. Continuous variables were analyzed with a 2-tail unpaired t test. Categorical variables were assessed with the χ 2 test or Fisher exact test. Logistic regression and multivariable logistic regression analysis with 95% CIs were used to obtain odds ratios. LGMS most commonly occurred in the oral cavity (49.2%). Half of all recurrences occurred in the paranasal sinuses/skull base. LGMS occurring at the paranasal sinuses/skull base had a significantly increased risk of recurrence compared with other subsites within the head and neck (odds ratio: -40; 95% CI: 2.190, 762.005; P = 0.013). The average time to recurrence of LGMS was 19.2 months. Adjuvant treatment with radiation did not improve recurrence rates. Sex, tumor size, or bony involvement were not found as risk factors for recurrence. Patients with LGMS of the paranasal sinuses and skull base are at high risk for recurrence and should be monitored closely. The role of adjuvant radiation treatment in these patients remains unclear.