welcome to oneFAPvoice- a positively charged Familial Adenomatous Polyposis community.
- join today!
Long-Term Outcome of Sporadic and FAP-Associated Desmoid Tumors Treated with High-Dose Selective Estrogen Receptor Modulators and Sulindac: A Single-Center Long-Term Observational Study in 134 Patients
source: Familial cancer
authors: Quast D R, Schneider R, Burdzik E, Hoppe S, Möslein Gsummary/abstract:
Aim of this study is to evaluate the outcome of long-term conservative treatment with sulindac and high-dose selective estrogen receptor modulators (SERMs) for sporadic and FAP-associated desmoid tumors. Desmoids are very rare tumors in the general population but occur frequently in FAP patients, being encountered in 23-38 %. Treatment of desmoids is still most controversial since response cannot be predicted and they are prone to develop recurrence. This study included all desmoid patients that were treated and followed at our institution and had completed at least 1 year of treatment. Response was defined as stable size or regression of desmoid size between two CT or MRI scans. A total of 134 patients were included. 64 (47.8 %) patients had a confirmed diagnosis of FAP, 69 (51.5 %) patients were sporadic. Overall 114 (85.1 %) patients showed regressive or stable desmoid size. Patients with previous history of multiple desmoid-related surgeries showed less-favorable response. The mean time to reach at least stable size was 14.9 (±9.1) months. After regression or stabilization, medication was tapered in 69 (60.5 %) of the treated patients with only one long-term recurrence after >10 years. The results of this study fortify the role of sulindac and high-dose SERMs as an effective and safe treatment for both, sporadic and FAP-associated desmoid tumors. While invasive treatment frequently results in high recurrence rates, high morbidity and high mortality, this conservative treatment is successful in most patients. The recurrence rate is negligible with no desmoid-related mortality in this large series. Therefore surgical resection, especially for mesenteric desmoids, should be deferred favoring this convincingly effective, well tolerated regimen.organization: HELIOS Klinikum Wuppertal
read more full text source
expertly curated content related to this topic
Victoria’s OTHER Secret: Designer Creates World’s first Lingerie Colostomy Bags as They often Put Partners OffA young designer has taken fashion into ...
Psychological Implications of Living with Familial Adenomatous PolyposisBACKGROUND AND STUDY AIMS: Psychosocial...
Inherited Colorectal Cancer Syndromes – Diagnosis and ManagemntColorectal cancer is the most common gas...
A Clinical Trial of COX and EGFR Inhibition in Familial Polyposis PatientsThe purpose of this study is to determin...
Pancreaticoduodenectomy for Advanced Duodenal and Ampullary Adenomatosis in Familial Adenomatous PolyposisBACKGROUND : Patients with familial aden...
A Serendipitous Diagnosis of Familial Adenomatous Polyposis. What Next?Poster Presentation Background: A sev...
Rectal Cancers in Patients with Familial Adenomatous PolyposisPatients with familial adenomatous polyp...