goserelin and Adenomatous-Polyposis-Coli

The optimal management of inoperable desmoid tumors is still unclear. We report a 26 year-old female patient with familial adenomatous polyposis suffering from a recurrent inoperable intraabdominal desmoid tumor and its sequential treatment.. Treatment strategies included low-dose tamoxifen (30 mg orally per day), high-dose tamoxifen (90 mg orally per day), and a subsequent combination of goserelin acetate (3.6 mg subcutaneously once every four weeks) plus low-dose tamoxifen, medroxyprogesterone acetate (1000 mg orally per day) and interferon gamma (3 Mio IU subcutaneously 3 times a week).. The combination of goserelin acetate and low-dose tamoxifen resulted in a decrease in tumour size and a complete relief of symptoms for 17 months. Thereafter the tumor progressed and again growth was stopped with interferon gamma therapy for another 6 months. All other treatment modalities had no effect.. This study demonstrates long-term regression of a desmoid tumor with combined endocrine therapy using goserelin acetate plus tamoxifen. Tumor progression after 17 months was again stopped by a combination of interferon-gamma and goserelin acetate.

Topics: Adenomatous Polyposis Coli; Adult; Antineoplastic Combined Chemotherapy Protocols; Drug Administration Schedule; Female; Fibromatosis, Aggressive; Goserelin; Humans; Interferon-gamma; Medroxyprogesterone; Mesentery; Neoplasm Recurrence, Local; Neoplasms, Multiple Primary; Peritoneal Neoplasms; Tamoxifen; Tomography, X-Ray Computed

Topics: Abdominal Neoplasms; Adenomatous Polyposis Coli; Adult; Aminopyridines; Antineoplastic Combined Chemotherapy Protocols; Cyclin-Dependent Kinase 4; Cyclin-Dependent Kinase 6; Female; Fibromatosis, Aggressive; Goserelin; Humans; Letrozole; Protein Kinase Inhibitors; Purines; Young Adult

Four patients are described with familial adenomatous polyposis and an intra-abdominal desmoid tumour located in the mesentery of the small bowel; the fifth patient had a tumour in the abdominal wall. In all patients a colectomy had been performed several years prior to the development of the tumours. Radical resection of the desmoid tumours was not possible because of the location in the mesentery; repeated resection or partial excision was performed in all four patients. NSAIDs, tamoxifen, norethisterone and interferon alpha appeared to have only a temporary effect on the growth of the desmoid tumour. The effect of goserelin, a gonadorelin analogue, on the behaviour of the desmoid tumour appears promising, as two patients treated with it showed no tumour growth after 4 and 16 months respectively.

Topics: Abdominal Neoplasms; Adenomatous Polyposis Coli; Adult; Combined Modality Therapy; Female; Fibroma; Gardner Syndrome; Goserelin; Humans; Male; Recurrence