Clinical features and long-term outcomes of aggressive fibromatosis of the abdominal wall after surgical resection: A retrospective study and literature review

Abstract

Aim: Aggressive fibromatosis of the abdominal wall is easily misdiagnosed prior to surgery as abdominal wall endometriosis or other tumors. The authors intend to evaluate the clinical features and outcomes of aggressive fibromatosis of the abdominal wall after surgical resection.

Methods: We retrospectively analyzed the data from 24 patients treated in the First Hospital of China Medical University from January 2011 to June 2019 and discussed the characteristic of this disease after a review of literature worldwide.

Results: Most patients were female (91.7%) with a mean age of 33 years (range 26-56 years). The most common symptom was progressive abdominal wall discomfort (ie, abdominal pain) in 16 patients (66.7%). Twelve patients (50%) had a history of cesarean section. All 24 patients underwent surgical R0 resection due to progression of disease or pain, including 12 patients treated with simple tumor resection and 12 patients treated with tumor resection followed by abdominal wall tension-free repair using synthetic mesh. Postoperative complications included incisional infection in three patients (12.5%), incisional hernia and postoperative bleeding in one patient each (4.2%). The local recurrence rate was 12.5%.

Conclusions: Aggressive fibromatosis of the abdominal wall mainly occurs in young women after cesarean section or other surgeries, with a high recurrence rate. Radical resection is an optimal treatment for the patients with progressive cases, and the restoration of extensive defects of the abdominal wall can be achieved with synthetic mesh, which leads to a good restoration of abdominal wall integrity.

Keywords: abdominal wall; aggressive fibromatosis; desmoid tumors; radical resection; tumor recurrence.