title: Treatment of mesenteric desmoid tumours with the anti-oestrogenic agent toremifene: case histories and an overview of the literature.
abstract: Desmoid tumours are histologically benign but due to their infiltration and compression of surrounding structures potentially life-threatening fibromatous lesions of unknown aetiology. The annual incidence rate is 2-4 per million people. The mesenteric variant constitutes about 10% of all desmoid tumours, although in familial adenomatous polyposis (FAP) patients this may be up to 70%. Due to the small number of patients with mesenteric desmoids the therapy is mainly empirical. This report describes the rationale as well as the value of the short- and long-term treatment (up to 6 years) with the anti-oestrogenic agent toremifene in combination with sulindac in two patients suffering from such a mesenteric desmoid tumour. These patients did not respond to sulindac alone and previous treatment with tamoxifen together with this non-steroidal anti-inflammatory drug had also failed. An overview of the literature on the management of these dismal tumours is presented.
authors.affiliation: Department of Gastroenterology-Hepatology, Leiden University Medical Center, The Netherlands.
authors.forename: P J
authors.initials: PJ
authors.lastname: Bus
authors.forename: H W
authors.initials: HW
authors.lastname: Verspaget
authors.forename: J H
authors.initials: JH
authors.lastname: van Krieken
authors.forename: A
authors.initials: A
authors.lastname: de Roos
authors.forename: H J
authors.initials: HJ
authors.lastname: Keizer
authors.forename: W A
authors.initials: WA
authors.lastname: Bemelman
authors.forename: H F
authors.initials: HF
authors.lastname: Vasen
authors.forename: C B
authors.initials: CB
authors.lastname: Lamers
authors.forename: G
authors.initials: G
authors.lastname: Griffioen
mesh_terms: D000328:Adult; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D005260:Female; D018221:Fibromatosis, Abdominal / Q000000981:diagnostic imaging / Q000188:drug therapy*; D006801:Humans; D008297:Male; D008643:Mesentery / Q000473:pathology*; D008875:Middle Aged; D012074:Remission Induction; D020845:Selective Estrogen Receptor Modulators / Q000627:therapeutic use*; D013467:Sulindac / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D017312:Toremifene / Q000627:therapeutic use*
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D020845:Selective Estrogen Receptor Modulators; D013467:Sulindac; D017312:Toremifene