Management of PEComas: A Review of the Role of Radiotherapy

Background/Objectives: Malignant PEComa is a rare sarcoma subtype and usually represents PEComa-NOS (not otherwise specified), one of the several entities of the PEComa family. Surgery is the primary treatment for localized disease; chemotherapy is used mainly for metastatic or unresectable cases. Radiotherapy (RT) may be considered in selected cases; however, its role remains unclear due to the rarity of the disease and limited radiotherapy-specific studies. Methods: This is a descriptive literature review of a limited number of reports on RT use in PEComa. Descriptive statistics were used to summarize reported case characteristics and outcomes. Results: We identified 28 publications reporting 33 cases. In neoadjuvant settings, there was a significant local response to RT in one case. In other neoadjuvant cases, although quantitative response assessments were not reported, most showed no recurrence during follow-up, with the longest follow-up at 34 months, suggesting that a possible benefit in local disease control may exist. In the adjuvant setting, some reports described prolonged disease-free survival following RT, though the lack of direct comparisons between surgery with versus without RT and heterogeneous follow-up periods limit definitive conclusions. In selected metastatic cases, palliative RT achieved notable local responses, potentially contributing to durable local control. Conclusions: In conclusion, although the only available data on RT in PEComas come from case studies with overall heterogeneous management approaches, RT has shown some potential as a therapeutic option across neoadjuvant, adjuvant, and palliative settings, warranting further dedicated clinical studies.