How hard fibroids are diagnosed and treated

Sclerofibrosarcoma, also known as invasive fibromatosis and ligamentous fibromatosis, is a rare tumor of fibroblastic origin. Sclerofibrosarcoma accounts for 0.03% of all tumors, with an annual incidence of 2-4 per million. The disease can occur in many parts of the body, with the scapular girdle, abdominal wall, lower extremities, pelvic girdle, trunk, upper extremities, head and neck, chest wall, and breast in that order. The average age of onset is about 30 years. The main risk factors for the development of hard fibroids are familial adenomatous polyposis, Gardner’s syndrome, trauma, and female. The clinical course of hard fibroids is variable and can be stable, grow aggressively, or regress spontaneously. Hard fibromas do not metastasize to lymph nodes and distant sites, and local recurrence is primarily due to its infiltrative growth characteristics rather than to satellite foci and jumping metastases. Malignancy is rare and may be associated with repeated surgical stimulation and radiation therapy. The clinical manifestation of sclerofibrosarcoma is usually a slow-growing, hard, fixed mass, sometimes with pain, and if the tumor is located near a joint, it may cause limitation of movement of the affected limb. MRI is the first choice for this disease because it can distinguish soft tissues well. MRI of hard fibroma is mainly a soft tissue mass growing along the muscle fibers, involving multiple muscles; the tumor is usually large with unclear borders; there is no cystic necrosis within the tumor and no edema around the tumor; T1WI is mostly isosignal or low signal, T2WI is mainly high signal with inhomogeneous signal; the tumor shows inhomogeneous enhancement after enhancement. The final diagnosis of hard fibroma must rely on pathology. Histologically, the tumor appears to be a normal-appearing fibroblast proliferation with rare mitosis and necrosis, and the tumor lacks pseudo-envelope and shows local infiltrative growth. It is generally accepted that surgery should be the first line of treatment for sclerofibroma. For patients with sclerofibroma, if surgery can preserve good function and appearance of the limb and achieve negative margins, only surgical resection should be performed and postoperative follow-up should be observed. However, the function and appearance of the limb should not be sacrificed in order to achieve negative margins, because those with positive margins can not only receive radiotherapy after surgery, but also have a second chance for surgery after recurrence. In addition, sclerofibroma is not life-threatening and therefore, unlike general malignancies, does not require radical resection at the expense of function. Radiotherapy should be the second line of treatment Patients with residual lesions under the microscope should be considered for adjuvant radiotherapy depending on the location and extent of the tumor. Patients with unresectable tumors or those whose resection would result in severe functional impairment should receive radiation therapy alone. However, many patients with sclerofibrosarcoma are young adults or children, and radiotherapy may cause certain delayed complications in this group, mainly including limb contracture, growth disturbance, and secondary malignancy. Therefore, radiotherapy should be chosen with caution for this population. Drug therapy is considered to be the third line of treatment after surgery and radiotherapy. Patients whose tumors are not amenable to surgery and/or radiotherapy can receive drug therapy. Drug therapy includes non-steroidal anti-inflammatory drugs, hormone therapy, interferon therapy, chemotherapy with anti-tumor drugs, targeted therapy, etc. Many new therapies such as chemical ablation therapy, limb perfusion chemotherapy, and preoperative neoadjuvant chemotherapy have also been reported.