[Intraabdominal desmoid tumors in familial adenomatous polyposis]

Gastroenterol Hepatol. 2013 Nov;36(9):580-6. doi: 10.1016/j.gastrohep.2012.12.005. Epub 2013 Mar 29.
[Article in Spanish]


Desmoid tumors are currently the main cause of morbidity and mortality in patients with familial adenomatous polyposis. More than 10% of these patients will develop these tumors during their lifetime and more than a third will suffer their consequences. The main risk factors for their development are female sex and abdominal surgery. The most frequent localization is intraabdominal. The therapeutic approach to these tumors has changed, and the surgical treatment of choice is currently the subject of debate. If a watch and wait approach is adopted, more than 50% of tumors will prove to be indolent. Therefore, the therapeutic strategy should be based on clinical presentation and should be decided by a multidisciplinary team working in a center with experience of these tumors. The present article proposes a prognostic classification to guide the therapeutic approach.

Keywords: Aggresive fibromatosis; Colorectal cancer; Cáncer colorrectal; Desmoid tumor; Familial adenomatous polyposis; Fibromatosis agresiva; Poliposis adenomatosa familiar; Tumor desmoide.

Publication types

  • English Abstract
  • Review

MeSH terms

  • Abdominal Neoplasms* / diagnosis
  • Abdominal Neoplasms* / epidemiology
  • Abdominal Neoplasms* / genetics
  • Abdominal Neoplasms* / pathology
  • Abdominal Neoplasms* / therapy
  • Adenomatous Polyposis Coli* / diagnosis
  • Adenomatous Polyposis Coli* / epidemiology
  • Adenomatous Polyposis Coli* / genetics
  • Adenomatous Polyposis Coli* / pathology
  • Adenomatous Polyposis Coli* / therapy
  • Adult
  • Age of Onset
  • Anti-Inflammatory Agents, Non-Steroidal / therapeutic use
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Disease Progression
  • Female
  • Fibromatosis, Aggressive* / diagnosis
  • Fibromatosis, Aggressive* / epidemiology
  • Fibromatosis, Aggressive* / genetics
  • Fibromatosis, Aggressive* / pathology
  • Fibromatosis, Aggressive* / therapy
  • Genes, APC
  • Humans
  • Male
  • Mutation, Missense
  • Neoplasm Staging
  • Point Mutation
  • Prognosis
  • Risk Factors
  • Sex Distribution
  • Watchful Waiting
  • Wnt Signaling Pathway
  • beta Catenin / genetics
  • beta Catenin / physiology


  • Anti-Inflammatory Agents, Non-Steroidal
  • CTNNB1 protein, human
  • beta Catenin

Supplementary concepts

  • Desmoid disease, hereditary