Sporadic desmoid tumors of the abdominal wall: the results of surgery

Tumori. 2016 Dec 1;102(6):582-587. doi: 10.5301/tj.5000552. Epub 2016 Sep 3.

Abstract

Purpose: Before the wait-and-see policy became the standard approach in abdominal wall desmoid tumors, surgery was performed on a systematic basis. Surgery remains indicated for progressing tumors but its extent is debatable. The abdominal wall is a common site of origin of sporadic desmoids, usually associated with a favorable prognosis. We analyzed the results of surgery at this specific site.

Methods: Data from 33 patients affected by sporadic desmoid tumors of the abdominal wall (31 primary, 2 recurrent) consecutively treated at our cancer center between January 2000 and September 2013 were retrospectively studied.

Results: Twenty-nine patients underwent surgery upfront and 1 after progression during the initial wait-and-see period. Prosthetic reconstruction of the abdominal wall was required in 28 patients. The average hospital stay was 5 days. Three patients developed surgical complications. Local recurrence-free survival was 90% at 5 and 10 years. Three patients had an uneventful childbirth during the follow-up after surgery.

Conclusions: Desmoid tumors of the abdominal wall have a favorable prognosis after surgical resection, which remains a safe and effective treatment. Wild-type tumors are common, whereas the incidence of S45F mutation in the beta-catenin gene is lower than in other anatomic sites. Upfront surgery may be considered in selected women who wish to bear a child.

MeSH terms

  • Adult
  • Disease Progression
  • Female
  • Fibromatosis, Abdominal / genetics
  • Fibromatosis, Abdominal / mortality
  • Fibromatosis, Abdominal / pathology*
  • Fibromatosis, Abdominal / surgery*
  • Humans
  • Male
  • Middle Aged
  • Mutation
  • Neoplasm Recurrence, Local
  • Postoperative Complications
  • Prognosis
  • Proportional Hazards Models
  • Treatment Outcome
  • Tumor Burden