ArticleFamilial cancer2015
Desmoid tumour in familial adenomatous polyposis patients: responses to treatments.
Article in Familial cancer, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers, 2 of them syntheses that pooled it.
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Who cites it
24 citing papers in PubMed, 2 syntheses or guidelines pooled it, 54 citations in OpenAlex.
- Updated European guidelines for clinical management of familial adenomatous polyposis (FAP), MUTYH-associated polyposis (MAP), gastric adenocarcinoma, proximal polyposis of the stomach (GAPPS) and other rare adenomatous polyposis syndromes: a joint EHTG-ESCP revision.The British journal of surgery · 2024Guideline
- Surgical management of abdominal desmoids: a systematic review and meta-analysis.Irish journal of medical science · 2023Pooled it
- Review
- 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
- Surgical Decision-Making in Familial Adenomatous Polyposis.Clinics in colon and rectal surgery · 2024Review
- A real-world study on the clinicopathological profile, treatment outcomes and health-related quality of life, anxiety and depression among patients with desmoid tumor at two tertiary care centers in India.Frontiers in oncology · 2024Article
- Desmoid Tumors in Familial Adenomatous Polyposis Patients: Favorable Outcomes with Multidisciplinary Management.Annals of surgical oncology · 2023Article
- Recent Advances and Current Management for Desmoid Tumor Associated with Familial Adenomatous Polyposis.Journal of the anus, rectum and colon · 2023Review
- Japanese Society for Cancer of the Colon and Rectum (JSCCR) guidelines 2020 for the Clinical Practice of Hereditary Colorectal Cancer.International journal of clinical oncology · 2021Article
- The Role of Radiation Therapy for Symptomatic Desmoid Tumors.Current treatment options in oncology · 2021Review
- Management of aggressive fibromatosis.Oncology letters · 2021Review
- Desmoid Tumors Characteristics, Clinical Management, Active Surveillance, and Description of Our FAP Case Series.Journal of clinical medicine · 2020Review
- Targeted therapy of desmoid-type fibromatosis: mechanism, current situation, and future prospects.Frontiers of medicine · 2019Review
- Recurrent aggressive mesenteric desmoid tumor successfully treated with sorafenib: A case report and literature review.World journal of clinical oncology · 2019Article
- Familial Polyposis Coli: The Management of Desmoid Tumor Bleeding.Open medicine (Warsaw, Poland) · 2019Article
- Familial Adenomatous Polyposis and Desmoid Tumor Treated with Multivisceral Transplantation and Kidney Autotransplantation: Case Report and Literature Review.Case reports in surgery · 2019Article
- Japanese Society for Cancer of the Colon and Rectum (JSCCR) Guidelines 2016 for the Clinical Practice of Hereditary Colorectal Cancer (Translated Version).Journal of the anus, rectum and colon · 2018Review
- The treatment of desmoid tumors associated with familial adenomatous polyposis: the results of a Japanese multicenter observational study.Surgery today · 2017Article
- Hereditary Colorectal Polyposis and Cancer Syndromes: A Primer on Diagnosis and Management.The American journal of gastroenterology · 2017Review
- Adult desmoid tumors: biology, management and ongoing trials.Current opinion in oncology · 2017Review
Corrections and comments
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Authors and funding
6 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
No guidelines for desmoid tumors (DT) management are available and DT have now become the first cause of death in FAP patients who had restorative proctocolectomy. We aimed to assess the results of the different treatments used for DT in Familial Adenomatous Polyposis (FAP) patients. All patients followed for FAP who developed a DT between 1970 and 2010 were collated. We analysed separately the history of DT according to location: mesenteric, parietal or mixed. 79 FAP patients [45 females (56 %); mean age 33.3 ± 12.5] presented 149 DT and were included; 16(20 %) had a DT diagnosed during or before first abdominal surgery and 47 (59 %) had isolated mesenteric DT. 11 patients had only surgical treatment, 17 only medical treatments, 31 had combined treatment and 20 had no treatment with spontaneous DT regression or stabilization. Overall, 80 treatment lines were administered to patients with a progression free or regression rate of 43 % (34/80). Response rates were: chemotherapy 77 % (10/13); Sulindac + tamoxifen 50 % (6/12); Tamoxifen 40 % (6/15); Imatinib 36 % (4/11); Sulindac 28 % (8/29). Among the 42 surgical procedures, an R0 resection was performed in 26 (62 %) allowing the absence of recurrence for 54 %. After a median follow-up of 81 months, 8 patients died of their DT and 6 died of other cause. Overall and DT-specific survival at 20 years were 52 and 79 %, respectively. Chemotherapy was the most efficient treatment. For intra-abdominal DT, we consider it as a first choice treatment and reserve surgery when it is impossible or when DT are life threatening.
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Registered trials
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