Background: Fibromatosis can be classified according to site of origin, namely, extraabdominal, abdominal wall, or intraabdominal. This study reports on the surgical management and long-term outcomes from a single center in the management of sporadic abdominal wall fibromatosis.
Methods: Patients who underwent surgery for abdominal wall fibromatosis between 1998 and 2013 were identified from a prospectively maintained database. A retrospective review of patient demographics, tumor characteristics, surgical outcomes, operative management, and recurrence rates was performed.
Results: Fifty patients underwent resection of a primary sporadic abdominal wall fibromatosis; 48 were female, of whom 43 reported previous pregnancy. Twenty-seven patients (54 %) had prior abdominal surgery for other pathologies. Macroscopic clearance was achieved in all cases. The median size of tumors resected was 8 cm (range 3-15 cm). The abdominal wall defect was reconstructed with prosthetic mesh in 47 of 50 cases. No major postoperative complication was encountered. Microscopic margins were reported as clear (R0) in 21 of 50 cases. With a median follow-up of 6 years (range 1-15 years), 46 of 50 patients remain disease free, with a median disease-free survival of 5 years. Of these 46 disease-free patients, 13 had further pregnancies without complications from either the abdominal mesh repair or tumor recurrence.
Conclusions: For asymptomatic sporadic abdominal wall fibromatosis, observation is an accepted first-line strategy. However, in contrast to extraabdominal fibromatosis, the preferred definitive treatment is surgical resection, which is recommended as first-line therapy in symptomatic patients, selected cases when tumors are progressing, and those with tumors >7 cm.