ArticleAnnals of surgical oncology2023
Desmoid Tumors in Familial Adenomatous Polyposis Patients: Favorable Outcomes with Multidisciplinary Management.
Article in Annals of surgical oncology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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Who cites it
6 citing papers in PubMed.
- Obstructive Pyelonephritis Secondary to Ureteral Compression by an Intra-Abdominal Desmoid Tumor in Familial Adenomatous Polyposis: A Case Report.Clinical case reports · 2026Article
- Management of desmoid tumors associated with familial adenomatous polyposis: a three-decade experience of a tertiary center in Brazil.Arquivos brasileiros de cirurgia digestiva : ABCD = Brazilian archives of digestive surgery · 2025Article
- Risk factors for postoperative recurrence of desmoid tumors: a retrospective cohort analysis.Frontiers in oncology · 2025Article
- Risk factors and protective measures for desmoid tumours in familial adenomatous polyposis: retrospective cohort study.BJS open · 2024Article
- Article
- Letter to the Editor on "Volume-based 18F-FDG-PET/CT predicts prognosis and outcome of active surveillance for intra-abdominal desmoid tumor".Abdominal radiology (New York) · 2024Article
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9 authors.
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Abstract
objectiveIn this study, we aimed to describe the clinical features, management, and outcomes of desmoid tumors (DTs) in familial adenomatous polyposis (FAP) patients at a high-volume sarcoma center.
methodsConsecutive patients with FAP and DTs were identified from our institutional databases (1985-2021). Patient demographics, treatment, and outcomes were described. Categorical data were compared using Fisher's exact test, and Kaplan-Meier curves were used to estimate progression-free survival (PFS).
resultsForty-five patients with 67 DTs were identified: 39 mesenteric or retroperitoneal (58.2%), 17 abdominal wall (25.4%), 4 extremity (6%), 4 breast (6%) and 3 back (4.4%). Severe DT symptoms were present in 12 patients (26.7%). Initial treatments per tumor were observation in 30 (44.8%) DTs, chemotherapy in 15 (22.4%) DTs, surgery in 10 (14.9%) DTs, and other systemic therapies in 10 (14.9%) DTs. The majority of DTs remained stable with observation or a single intervention (77.8%). Median PFS was 23.4 years (95% confidence interval 7.6-39.2). In the 12 severely symptomatic patients, four patients required more than two interventions for DT control. At a median follow-up of 6.0 years (range 0.7-35.8 years), 33 (73.3%) patients were alive with disease, 7 (15.6%) were alive without disease, and 5 (11.1%) died of other causes. No patients died of DT-related complications.
conclusionsThe majority of DTs in FAP patients remained stable with observation or a single intervention. There were no DT-related deaths; however, 12 of 45 patients (26.7%) experienced significant tumor morbidity and required more interventions for disease control. Further studies on quality of life are required.
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