关键词: 腹壁硬纤维瘤, 主动监测, 靶向药物

Abstract: Advancements in the diagnosis and treatment of abdominal wall desmoid tumors        LIU Bai-qi, HUANG Geng-wen. Division of Hernia and Abdominal Wall Surgery, Department of General Surgery, Xiangya Hospital, Central South University, Changsha 410008, China
Corresponding author: HUANG Geng-wen, E-mail: huanggengwen@csu.edu.cn
Abstract    Abdominal desmoid tumors (DT) are a type of invasive fibroma that occur in the abdominal wall muscle layer. With a low incidence rate and limited understanding clinically, these tumors are characterized by local infiltration, high recurrence rate, and an unpredictable disease course. The understanding and treatment of this disease are constantly improving and strategies are being optimized. Diagnostic methods include ultrasound, CT, and MRI. The specific imaging manifestations of DT require differentiation from inflammatory diseases or malignant soft tissue tumors. A final diagnosis is confirmed through biopsy and histopathology. Active surveillance (AS) is a current first-line treatment strategy. For patients with slow growth, no symptoms, or high surgical risk, AS is an effective way to avoid overtreatment. For abdominal DT that requires treatment intervention, surgical treatment is the first choice. Positive-margin postoperative or inoperable abdominal DT may be treated with drug therapy or radiotherapy. Furthermore, all abdominal DT patients should be fully evaluated and treated by a sarcoma multidisciplinary team, and undergo regular follow-up and imaging examinations. This will help evaluate treatment effects and recurrence, adjusting treatment plans as necessary. For pregnant DT patients, AS remains the first choice treatment. For patients with fast-growing DT, aggressive surgical treatment during pregnancy is safe and effective. However, antitumor drugs can cause adverse events related to ovarian dysfunction, potentially affecting the fertility of women of childbearing age. Therefore, potential fertility risks should be fully understood before treatment, and consultation with the reproductive clinic is recommended. Even though understanding of DT has improved in recent years, there is still no uniform standard for diagnosing and treating abdominal DT. Individualized treatment plans should be customized according to each case.

Key words: abdominal wall desmoid tumor, active surveillance, targeted drugs