Pharmacologic treatment of desmoid tumors in familial adenomatous polyposis: results of an in vitro study.
BACKGROUND
Desmoid tumors (DTs), the result of an abnormal proliferation of connective tissue, occur frequently in familial adenomatous polyposis. Treatment of DT is difficult because of the high rate of recurrence after operation. Recently, antiestrogens and nonsteroidal antiinflammatory drugs have been used with good results as inhibitors of DT cell proliferation.


METHODS
In this report we performed in vitro studies on cultured desmoid cells and skin fibroblasts of four patients who underwent surgical resection of DT and normal skin biopsy. We evaluated the expression of estrogen receptors and the mitogenic effect of 17 beta-estradiol and sulindac, a nonsteroidal antiinflammatory compound, on cell proliferation and collagen synthesis of desmoid cells.


RESULTS
Proliferation and collagen synthesis of desmoid cells were stimulated by 17 beta-estradiol, and tamoxifen, an antiestrogenic compound, inhibited this effect. Desmoid cells also expressed estrogen receptors. Moreover, growth of desmoid cells from one of the patients was inhibited by sulindac.


CONCLUSIONS
The in vitro evaluation of drug responsiveness in patients operated on for DT could be used as both a prognostic tool in the natural history of DT and in addressing pharmacologic therapy in this disorder.
Tonelli
F
F
Department of Clinical Physiopathology, University of Florence, Italy.
Valanzano
R
R
Brandi
M L
ML
D011125:Adenomatous Polyposis Coli / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology; D000293:Adolescent; D000328:Adult; D002455:Cell Division / Q000187:drug effects; D002648:Child; D003094:Collagen / Q000096:biosynthesis; D004958:Estradiol / Q000494:pharmacology; D018222:Desmoid Tumors / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology; D006801:Humans; D011960:Receptors, Estrogen / Q000378:metabolism; D013467:Sulindac / Q000494:pharmacology; D014407:Tumor Cells, Cultured
D011960:Receptors, Estrogen; D013467:Sulindac; D004958:Estradiol; D003094:Collagen