Aggressive fibromatosis (AF) has many different names and is customarily called ligamentous fibromatosis. It is a rare tumor of fibrous tissue origin, accounting for 1.19% of fibrous tissue tumors. It is a rare tumor of fibrous tissue origin, accounting for 1.19% of fibrous tissue tumors. It is pathologically benign, but clinically aggressive and recurrent, but does not metastasize. Currently, surgery is the main treatment for this disease, and radiotherapy and hormonal therapy also have a role, but the results are unsatisfactory, so it is necessary to re-evaluate this type of lesion. 1. Epidemiology The disease was first described by Mc Farlane in 1832 and was officially named by Muller in 1838. Scholars at home and abroad have conducted in-depth studies on it and agreed that the incidence of this disease is very low, accounting for 0.03% of soft tissue tumors, but the incidence of familial adenomatous polyposis is as high as 8%-38%, which is 852 times higher than that of the general population. The etiology of adenomatous polyposis is not completely clear, but may be related to trauma, endocrine and defective growth regulation of connective tissue. In familial adenomatous polyposis, about 80% to 90% of the tumors are located in the small intestine mesentery and abdominal wall, and the age is 25 to 30 years old. Sclerofibrosarcoma is sometimes associated with pain and local swelling, especially when it invades the adjacent neurovascular and joints. The diagnosis mainly relies on pathological examination, but it is worth noting that fine needle aspiration biopsy is not very helpful due to the lack of cytologic integrity of the material taken, and MRI has a reference value. What is seen with the naked eye:The tumor is of different sizes, without envelope, with irregular margins, tough texture like rubber, grayish-white cut surface and woven arrangement of fiber bundles, often invading surrounding tissues, such as muscle and fat inside. Microscopically, the tumor is composed of well-differentiated fibroblasts and collagen fibers, and the fibroblasts are large, with long nuclei, lightly stained, and one to three nucleoli. The tumor cells often invade the surrounding tissues, and the muscle fiber tissues are separated into small islands and undergo atrophy and degeneration, and multinucleated muscle giant cells can be seen. 4.Treatment (1) Surgery Surgery has been the first line of treatment for AF patients, even though there are times when surgery may affect the aesthetics and function of the affected area, the side effects of surgery may seem insignificant compared to the organismal harm caused by AF. The main factor affecting the prognosis is the incision margin. The general surgical principle is to try to achieve a negative margin of 1-5 cm to avoid recurrence, provided that the function is protected. Nuyttens et al. also showed that a combination of surgery and radiotherapy can greatly improve the cure rate, regardless of whether the margin is negative or positive. Many studies have reported risk factors for local recurrence, including tumor diameter greater than 4 cm, inadequate margins, age at presentation less than 32 years, and extra-abdominal AF. Most of the reports support the treatment plan of surgery plus radiotherapy. (2) Radiotherapy for AF is mainly applied to patients with unresectable, incompletely resected or positive margins, and Ewing suggested as early as 1928 that the response of sclerofibrosarcoma to radiotherapy is “slow but effective”. Many recent studies have also shown a definite therapeutic effect of radiotherapy alone and adjuvant radiotherapy after surgery. The University of Florida College of Medicine achieved excellent results in 65 patients treated with AF radiation therapy, while additional surgery did not significantly improve their outcomes. Radiotherapy did not reduce the recurrence rate, but prolonged the recurrence time. Analysis shows that there is no significant difference in the treatment effect between radiation therapy doses of 50-60 Gy and those above 60 Gy, but the side effects increase significantly when the radiation therapy dose exceeds 60 Gy, especially in patients who have undergone osteotomy, bone scraping or other procedures affecting lymphatic drainage. The side effects include slow tissue growth, fibrosis, edema, skin ulcers, cellulitis, pathological fractures, secondary tumors, and neurological lesions including paresthesia and sensory abnormalities. (3) Chemotherapy Since familial adenomatous polyposis (FAP) patients have a 3.5% to 32% incidence of AF, and surgery is not able to help, this makes drug therapy more important. Currently, NSAIDs and anti-estrogen drugs are the first-line drugs for this treatment, while cytotoxic chemotherapeutic agents are mostly used when surgery, radiotherapy, NSAIDs and anti-estrogen drugs are ineffective. Since NSAIDs have been shown to reduce the number of polyps in the GI tract of FAP patients, it is inferred that they should also be effective in DT with the same pathogenesis. Currently the most used NSAID for DT is sulforaphane, but there are no large randomized studies on its efficacy. Given the observation that premenopausal women have a higher incidence of DT than postmenopausal women and men, and that their DT is more aggressive, anti-estrogenic drugs are now one of the mainstays of DT treatment. Raloxifene and tamoxifen have been used more frequently, and other drugs such as cAMP inhibitors, alpha interferon, and the antifibrotic drug pirfenidone have been used, but all of them lack bulk randomization studies.