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

Abstract

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.

Value of Porous Titanium Alloy Plates for Chest Wall Reconstruction after Resection of Chest Wall Tumors

“…However, the resection range should be properly enlarged to such tumors as desmoids, chondroma and osteoclastoma, or be resolved as in malignant tumors, though with benign pathological examinations (Errani et al, 2010;Nieuwenhuis et al, 2011). Chest wall resection can bring about different degrees of chest wall defects, which need to be repaired and reconstructed (Munhoz et al, 2011;Maeda et al, 2014).…”

A Systematic Review of Active Treatment Options in Patients with Desmoid Tumours

“…Fifteen articles that did not provide clear comparative data for each group were excluded from further analysis [33][34][35][36][37][38][39][40][41][42][43][44][45][46][47] .…”

Diagnosis and multidisciplinary treatment of sporadical desmoid tumors

“…Radical excision is often a therapeutic challenge or even impossible for intra-abdominal desmoids, confirmed by previous and recent studies which reported a rather low success rate of radical removal of intra-abdominal or retroperitoneal desmoids (8,32,91,107,(113)(114)(115)(116).…”

“…The optimal therapy for desmoids is difficult to be established due to the heterogeneous clinical behavior and anatomical presentation associated to the variable etiology, the high local recurrence rate, the infilltrative growth and the rarity of the tumor (8,15,91). There is limited or no evidence based medicine supporting treatment choice (5,7,8,15,85 …”

“…nodular fasciitis, hypertrophic scars, keloids) to fibroblastic sarcomas (2, 7). Histologically, desmoids are often indistinguishable from nonneoplastic lesions associated with abnormal wound healing and differentiation from other soft tissue tumors, such as fibromyxoid sarcomas, can be challenging (33,91,92 …”