Shon et al consider lymphedema as a significant risk factor for the development of angiosarcoma.14In total, 65 cases of MLL were described by 2015, and 9 (10.3%) of them progressed to angiosarcoma.15According to Best and coauthors, MLL is a benign tumor, the excision of which is desirable because it can progress to angiosarcoma in 13%.16Other authors also noted the malignization of MLL.11, 14The mortality rate from MLL is 9% that justifies oncological alertness while diagnosing MLL.13, 14, 17. The gene discussed is KMT2A; the disease is lymphedema.