ABSTRACT
Actualmente los tumores desmoides constituyen la principal causa de morbimortalidad en los pacientes con PAF. Más del 10% de estos pacientes desarrollarán estos tumores a lo largo de su vida, y más de un tercio de ellos sufrirán sus consecuencias. Los principales factores de riesgo para su desarrollo son el sexo femenino y el traumatismo de la cirugía abdominal, y la localización más frecuente es la intraabdominal. El enfoque terapéutico de estos tumores ha evolucionado y el tratamiento quirúrgico de primera línea se encuentra actualmente bajo debate. Se ha demostrado que si se opta por una estrategia de «esperar y ver», más del 50% de los pacientes tienen un curso indolente. Por lo tanto, la estrategia terapéutica debe basarse en la presentación clínica, en un contexto multidisciplinario y en un centro con experiencia en este campo. En este artículo se propone una clasificación pronóstica que guía el enfoque terapéutico (AU)
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 approachis 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 aprognostic classification to guide the therapeutic approach (AU)
Subject(s)
Humans , Fibroma, Desmoplastic/pathology , Abdominal Neoplasms/pathology , Adenomatous Polyposis Coli/pathology , Colorectal Neoplasms/pathologyABSTRACT
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.
