| Summary: | Lan Liu,1 Meizuo Zhong,1,2 Xuan Zhou,3 Fanhua Kang,4 Yong Long,1 Junfeng Li1 1Department of Oncology, Xiangya Changde Hospital, Changde, Hunan, People’s Republic of China; 2Department of Oncology, Xiangya Hospital, Central South University, Changsha, Hunan, People’s Republic of China; 3Department of Hematology, Xiangya Changde hospital, Changde, Hunan, People’s Republic of China; 4Department of Pathology, Xiangya Changde Hospital, Changde, Hunan, People’s Republic of ChinaCorrespondence: Junfeng Li, Email 430723461x@163.comAbstract: Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive malignancy. Most patients are diagnosed at a late stage with poor prognosis. The treatment usually includes combined intensive chemotherapy, cytoreductive surgery, radiotherapy, and targeted therapy. Due to the low incidence rate and dismal survival, there is currently a lack of case reports on DSRCT with concurrent leukemia. We report a case of a young patient who achieved disease stabilization for 14 months after receiving 6 cycles of chemotherapy and whole abdominal radiation therapy (WART), followed by consolidation treatment with anlotinib. However, the treatment was terminated due to the development of Acute Myeloid Leukemia-M5 (AML-M5). Multimodal therapy may provide a survival benefit for rare tumors that lack standard treatment. However, intensive chemotherapy and extensive radiotherapy carry a risk of inducing secondary malignancies. This is the first reported case of concurrent DSRCT and AML-M5 with short intervals between onset.Keywords: desmoplastic small round cell tumor, whole abdominal radiation therapy, anlotinib, therapy-related acute myeloid leukemia
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