Evaluation of management of desmoid tumours associated with familial adenomatous polyposis in Dutch patients

• Published in: British journal of cancer Vol. 104; no. 1; pp. 37 - 42
• Main Authors: Nieuwenhuis, M H, Mathus-Vliegen, E M, Baeten, C G, Nagengast, F M, van der Bijl, J, van Dalsen, A D, Kleibeuker, J H, Dekker, E, Langers, A M, Vecht, J, Peters, F T, van Dam, R, van Gemert, W G, Stuifbergen, W N, Schouten, W R, Gelderblom, H, Vasen, H F A
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 04.01.2011; Nature Publishing Group
• Subjects: 692/308/409; 692/699/67/1504/1885; 692/700/565/1436; 692/700/565/545/546; Adenomatous Polyposis Coli - complications; Adenomatous Polyposis Coli - therapy; Adolescent; Adult; Antineoplastic Agents - therapeutic use; Biological and medical sciences; Biomedical and Life Sciences; Biomedicine; Cancer Research; Clinical Study; Colectomy; Combined Modality Therapy; Drug Resistance; Epidemiology; Female; Fibromatosis, Abdominal - complications; Fibromatosis, Abdominal - therapy; Fibromatosis, Aggressive - complications; Fibromatosis, Aggressive - therapy; Gastroenterology. Liver. Pancreas. Abdomen; Humans; Incidence; Male; Medical sciences; Middle Aged; Molecular Medicine; Netherlands; Oncology; Radiotherapy Dosage; Retrospective Studies; Stomach. Duodenum. Small intestine. Colon. Rectum. Anus; Survival Rate; Treatment Outcome; Tumors; Young Adult; Netherlands; Europe; desmoid-type fibromatosis; familial adenomatous polyposis; management; desmoid tumour; Pseudotumor; Human; Familial adenomatous polyposis coli; Desmoid tumor; Genetic disease; Fibromatosis; Cancerology; Clinical management; Digestive diseases; Intestinal disease; Benign neoplasm
• ISSN: 0007-0920; 1532-1827
• DOI: 10.1038/sj.bjc.6605997

Background: The optimal treatment of desmoid tumours is controversial. We evaluated desmoid management in Dutch familial adenomatous polyposis (FAP) patients. Methods: Seventy-eight FAP patients with desmoids were identified from the Dutch Polyposis Registry. Data on desmoid morphology, management, and outcome were analysed retrospectively. Progression-free survival (PFS) rates and final outcome were compared for surgical vs non-surgical treatment, for intra-abdominal and extra-abdominal desmoids separately. Also, pharmacological treatment was evaluated for all desmoids. Results: Median follow-up was 8 years. For intra-abdominal desmoids ( n =62), PFS rates at 10 years of follow-up were comparable after surgical and non-surgical treatment (33% and 49%, respectively, P =0.163). None of these desmoids could be removed entirely. Eventually, one fifth died from desmoid disease. Most extra-abdominal and abdominal wall desmoids were treated surgically with a PFS rate of 63% and no deaths from desmoid disease. Comparison between NSAID and anti-estrogen treatment showed comparable outcomes. Four of the 10 patients who received chemotherapy had stabilisation of tumour growth, all after doxorubicin combination therapy. Conclusion: For intra-abdominal desmoids, a conservative approach and surgery showed comparable outcomes. For extra-abdominal and abdominal wall desmoids, surgery seemed appropriate. Different pharmacological therapies showed comparable outcomes. If chemotherapy was given for progressively growing intra-abdominal desmoids, most favourable outcomes occurred after combinations including doxorubicin.