目的 探讨胰腺恶性纤维组织细胞瘤的诊断及治疗。方法 对兰州大学第一医院收治的1例胰腺恶性纤维组织细胞瘤患者，结合国、内外文献对其临床特点，影像学、病理学及免疫组化特征、治疗和预后进行分析。结果 行胰腺肿瘤切除，术后医用直线加速器采取三维适形精确放疗，随访9个月，未见肿瘤复发。结论 胰腺恶性纤维组织细胞瘤恶性度高，易复发、转移，生存率低，诊断主要依靠病理和免疫组化检查。外科手术联合放射治疗可延长生存时间和延缓肿瘤的复发。
ObjectiveTo summarize the experience of diagnosis and treatment of a case of adult hepatic fibrohistiocytoma with recurrent rupture and hemorrhage.MethodsRetrospective analysis on this case of recurrent adult fibrohistiocytoma with rupture and hemorrhage admitted to Shengjing Hospital Affiliated to China Medical University in May 2018 was conducted, discussion on MDT was conducted before surgery, and the causes of recurrent rupture and hemorrhage, as well as the experience of diagnosis and treatment were summarized and analyzed.ResultsThis patient was generally in good condition. After MDT discussions among the imaging department, oncology department, interventional department, and hepatobiliary surgeons, it was considered that surgical treatment was the best treatment scheme. After full communication with the patient and his family, the patient had chosen open tumor resection and the operation was smooth. The operation time was 278 min, intraoperative bleeding was about 100 mL, and no blood transfusion was performed. No bleeding, abdominal infection, gastroparesis, and other complications occurred after the operation, and the patient was discharged successfully on the 13th day after the operation. The patient had been interviewed for 8 months and the tumor recurred again 1 month ago. Currently, oral apatinib had been targeted for treatment for more than 1 month, and the follow-up treatment continued.ConclusionsRecurrent adult fibrous histiocytoma with rupture and hemorrhage is rare in clinical practice, and the treatment is mainly based on surgical resection. Postoperative comprehensive treatment combined with radiotherapy, chemotherapy, molecular targeting, and immunotherapy is the main treatment. Its short-term efficacy is satisfactory, but long-term efficacy still needs to befurther observed.