Liu Lan, Zhong Meizuo, Zhou Xuan, Kang Fanhua, Long Yong, Li Junfeng
Department of Oncology, Xiangya Changde Hospital, Changde, Hunan, People's Republic of China.
Department of Oncology, Xiangya Hospital, Central South University, Changsha, Hunan, People's Republic of China.
Onco Targets Ther. 2024 Feb 27;17:163-169. doi: 10.2147/OTT.S434286. eCollection 2024.
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.
促纤维组织增生性小圆细胞肿瘤（DSRCT）是一种罕见且侵袭性很强的恶性肿瘤。大多数患者在晚期被诊断出来，预后较差。治疗通常包括强化联合化疗、减瘤手术、放疗和靶向治疗。由于发病率低且生存率不佳，目前缺乏关于DSRCT并发白血病的病例报告。我们报告一例年轻患者，在接受6个周期的化疗和全腹放射治疗（WART）后，疾病稳定了14个月，随后用安罗替尼进行巩固治疗。然而，由于急性髓系白血病-M5（AML-M5）的发生，治疗终止。多模式治疗可能为缺乏标准治疗的罕见肿瘤提供生存益处。然而，强化化疗和广泛放疗有诱发继发性恶性肿瘤的风险。这是首次报道的DSRCT与AML-M5并发且发病间隔短的病例。
