Low-grade malignant spindle cell histofibrosarcoma is a kind of soft tissue malignant tumor whose etiology is not clear at present, which is clinically prevalent in long bone tissues, mainly manifested as local pain and soft tissue swelling, and the main treatment is surgical resection, which is supplemented by chemotherapy after surgery and is not easy to recur, with a fair prognosis. Malignant spindle cell histofibrosarcoma belongs to the category of soft tissue sarcoma, which is a kind of common soft tissue malignant tumor, this kind of tumor is most common in middle-aged and old people, and the etiology and pathogenesis of this kind of tumor are not clear at present. This kind of tumor tends to grow in the long bone tissues of human body such as femur and humerus, and many patients consult the doctor due to the pain of the bone at the local lesion and the swelling of the soft tissues around the bone. Once found, extensive radical surgery should be given as early as possible, and chemotherapy should be given in order to reduce the recurrence rate after the surgery, so as to avoid the delay of the disease.