Neurofibrosarcoma is a relatively rare tumor, accounting for 5% to 10% of malignant soft tissue tumors. The age of onset is more common in youth and middle age. The prevalent sites are mostly in the head and neck, buttocks, limbs and retroperitoneum, but also in the back, abdominal wall and mediastinum. Some cases have pain in the affected limb first, and then the mass and the affected neurogenic dysfunction appear. Localized pressure is painful, and neurofibromatosis is associated in 40% of cases. Because of the mild symptoms, most of the tumors are larger when they come to the clinic, and most of them are more than 10 cm in length and diameter. Pathologic changes: The typical tumor has a large nerve trunk attached to it, but it is a neurofibrosarcoma with no nerve, so there is no nerve to be found, and the lesion is deeper, and like other sarcomas, the cut surface is grayish white, and may be accompanied by hemorrhage and necrosis. Microscopically, malignant Schwann’s cells can be seen, with spindle-shaped tumor cells, large deep-stained nuclei, uneven size, and visible nuclear fission images, and epithelial-like Schwann’s cells, with round and multi-deformed tumor cells, variable cytoplasm, and pink-stained granules, arranged in solid nest foci, or in glandular or striated structures. The main treatment is wide excision, and amputation is feasible for those who have recurrence and are difficult to perform wide excision, and radiotherapy can be used for those who cannot perform wide excision or incomplete excision in the pelvis and paravertebral area, but it is not effective. There are reports in the literature that chemotherapy is effective for tumors with metastases, and commonly used drugs include adriamycin, vincristine, cyclophosphamide, etc. Hematogenous and lymphatic metastases can occur, and the 5-year survival rate is about 30%.