HighlightsThe most definitive feature of LMS is immunohistochemical positivity for desmin and SMA, and negativity for DOG 1, CD34, S-100 protein, and c-kit.It is crucial to diagnose leioyosarcoma precisely based on immunohistochemistry, and thereby distinguish it from GIST.Lymphadenectomy appears to be important for high-risk LMSs to perform R0 resection.