尤文肉瘤继发骨髓增生异常综合征继而急髓变1例

A Case of Ewing Sarcoma Secondary to Myelodysplastic Syndrome Followed by Acute Myeloid Transformation

摘要: 目的：探讨尤文肉瘤继发骨髓增生异常综合征继而急髓变的发病原因及诊治特点。方法：报道1例尤文肉瘤继发骨髓增生异常综合征继而急髓变，分析其临床特点及诊疗经过，复习国内外相关文献。结果：一名4岁10月男孩确诊左股骨尤文肉瘤，结束化疗和放疗3年5月后又确诊骨髓增生异常综合征，经过化疗和异基因造血干细胞移植后好转，移植后1年3月后又确诊急性髓系白血病，经过化疗和索拉非尼靶向治疗缓解后又早期复发，拟行第二次造血干细胞移植。结论：本患儿继发第二恶性肿瘤可能与化疗、放疗均有关，最终患儿可能预后不佳。

Abstract: Objective: To investigate the pathogenesis, diagnosis and treatment of Ewing’s sarcoma with secondary myelodysplastic syndrome and then acute myeloid transformation. Methods: A case of Ewing sarcoma secondary to myelodysplastic syndrome with acute myeloid transformation was reported, and its clinical features and diagnostic and therapeutic procedures were analyzed, and the relevant domestic and international literature was reviewed. Results: A 4-year-old boy was diagnosed with Ewing sarcoma of the left femur at the age of 10 months, and was diagnosed with myelodysplastic syndrome 3 years and 5 months after the end of chemotherapy and radiotherapy, and improved after chemotherapy and allogeneic hematopoietic stem cell transplantation, and then diagnosed with acute myeloid leukemia 1 year and 3 months after transplantation, and then relapsed at an early stage after remission from chemotherapy and sorafenib targeted therapy, and was proposed to undergo a second hematopoietic stem cell transplantation. Conclusion: The secondary second malignancy in this child may be related to chemotherapy and radiotherapy, and the final prognosis of the child may be poor.