Mortality of Intra-Abdominal Desmoid Tumors in Patients With Familial Adenomatous Polyposis

Abstract

Our study confirmed the validity of the staging system to predict mortality in patients with intra-abdominal desmoid tumors and identified additional risk factors able to better define the risk of death within each stage. Risk stratification is crucial in directing patients with advanced disease and poor prognosis to the most appropriate medical and surgical options.

Robotic-Assisted Distal Pancreatectomy for a Pancreatic Desmoid Tumor: A Case Report and Review of the Literature

“…They are slow growing and usually present with abdominal pain as was the case with our patient, or are incidentally discovered during imaging done for other reasons [16]. While most DTs are slow growing, non-metastatic, locally infiltrating and either compress or obstruct surrounding structures and are amenable to resection, some tumors are fast growing and cause bowel perforation, bleeding and bowel ischemia by invading the mesenteric blood supply [17,18]. This tumor behavior has been historically labelled as "locally malignant" [4].…”

Desmoid abdominal tumour: a clinical case report and brief literature review

“…10-25% of patients with FAP develop DT and these neoplasms are important cause for mortality after total colectomy. 7 Most sporadic DT (85%) have specific point mutations in exon 3 of gene coding beta-catenin (cadherin-associated proCase Report tein beta-1, CTNNB1), 8 therefore betacatenin signaling may have an impact on DT growth and recurrence. 9,10 Clinical presentations of abdominal DT includes local pain, palpable mass or local complications, like obstruction of the bowels, intestinal ischemia, fistulization, urethral compression with subsequent obstructive nephropathy.…”

Intra-abdominal fibromatosis with vesico-colonic fistula: case of a benign disease running a malignant course

“…15 Patients with FAP and intra-abdominal desmoids tend to have more severe disease and increased recurrence rates. Even if colonoscopy is initially normal, these patients are recommended for routine surveillance with colonoscopy.…”