Desmoid or aggressive fibromatosis is a rare soft tissue tumor. This entity accounts for less than 3% of soft-tissues tumours or 0.03% of all neoplasms and is more common in women. Its peak incidence is between 25 and 35 years of age, and most cases occur between puberty and 40 years of age. Many studies have shown that between 37% and 50% of desmoids tumors arise in the abdominal region. For those cases localized in the anterior abdominal wall radical resection and reconstruction with a mesh is indicated. Although randomized trials are lacking, in the early reported retrospective series, local recurrence was not uncommon. It has been recently proved that wide surgical excision and immediate plastic reconstruction with mesh after intraoperative confirmation by frozen section of disease-free margins >1 cm is a safe procedure and can provide a definitive cure for this disease. Long-term results after wide excision are quite encouraging with low recurrence rates, without functional and aesthetic limitations. For the management of the most complex cases with huge tumors a multidisciplinary surgical team comprising the general and the plastic surgeon is advocated. In recurrent cases or patients with poorly-located lesions, when a massive demolition of the abdominal wall is required, a wait and see approach could be a viable option, especially in asymptomatic patients, as well as medical therapy, mainly with COX-2 inhibitors and/or Tamoxifen.
|Title of host publication||Abdominal Wall Defects: Prevalence, Surgical Management Strategies and Clinical Care Outcomes|
|Publisher||Nova Science Publishers, Inc.|
|Number of pages||13|
|ISBN (Print)||9781629487106, 9781629486727|
|Publication status||Published - Jan 1 2014|
ASJC Scopus subject areas
- Social Sciences(all)