toremifene and Fibromatosis--Abdominal

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.

Topics: Adult; Anti-Inflammatory Agents, Non-Steroidal; Female; Fibromatosis, Abdominal; Humans; Male; Mesentery; Middle Aged; Remission Induction; Selective Estrogen Receptor Modulators; Sulindac; Tomography, X-Ray Computed; Toremifene

To report a case of a pelvic desmoid tumor that was treated with the antiestrogen toremifene after a failed attempt at surgical excision.. Case report.. University reproductive endocrine practice.. A reproductive-aged woman with a recurrent desmoid tumor.. After surgical excision of a desmoid tumor that presented during childbirth, subsequent recurrence resulted in the use of toremifene for tumor stabilization.. Magnetic resonance imaging was used to monitor desmoid tumor size.. One year after postsurgical recurrence of the desmoid tumor, the patient began treatment with the antiestrogen toremifene. Tumor stabilization and regression with symptomatic relief was observed. Nine years of antiestrogen use revealed no progression in tumor size or patient symptoms. After the patient demonstrated perimenopausal symptoms, toremifene administration was discontinued without a return of symptoms or tumor growth after 3 years.. Our case demonstrates that toremifene is a safe and effective therapy that can be used for the stabilization and regression of desmoid tumors. An antiestrogen should be considered as adjuvant therapy after surgery and as a first-line treatment with disease recurrence. Discontinuation of antiestrogen therapy was shown to be done safely after the patient started to show signs of decreased endogenous estrogen production.

Topics: Adult; Estrogen Antagonists; Female; Fibromatosis, Abdominal; Fibromatosis, Aggressive; Humans; Neoplasm Recurrence, Local; Toremifene

Tamoxifen and toremifene have been used in patients with advanced desmoid tumours with response rates of 51%.. We developed an experimental model of desmoid tumour cells in tissue culture to study their effect. Four cell lines were established in tissue culture. All native and corresponding cultured tumours were oestrogen receptor negative. Tumour 1 was from a 22 year old with familial adenomatous polyposis (FAP) and recurrent abdominal wall desmoid tumours. She remains disease free on tamoxifen 4 years following surgery. Both her mother and sister also have shown regression of their FAP-associated desmoid tumours at the menopause and on tamoxifen, respectively. We assessed the effect of tamoxifen on desmoid tumours in tissue culture at 780 ng/mL. The results were assessed by cell density counting.. Tumours 1 and 2 have responded with an approximately. 50% reduction in growth to tamoxifen at 780 ng/mL.. This apparent growth inhibitory effect of tamoxifen on two desmoid tumour cell lines appears to be independent of oestrogen and correlates with the in vivo effect of tamoxifen on three desmoid tumours in an FAP family.

Topics: Abdominal Neoplasms; Adenomatous Polyposis Coli; Adult; Estrogen Antagonists; Female; Fibromatosis, Abdominal; Humans; Male; Middle Aged; Receptors, Estrogen; Tamoxifen; Toremifene; Tumor Cells, Cultured