Treatment of mesenteric desmoid tumours with the anti-oestrogenic agent toremifene: case histories and an overview of the literature.
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.
Bus
P J
PJ
Department of Gastroenterology-Hepatology, Leiden University Medical Center, The Netherlands.
Verspaget
H W
HW
van Krieken
J H
JH
de Roos
A
A
Keizer
H J
HJ
Bemelman
W A
WA
Vasen
H F
HF
Lamers
C B
CB
Griffioen
G
G
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*
D000894:Anti-Inflammatory Agents, Non-Steroidal; D020845:Selective Estrogen Receptor Modulators; D013467:Sulindac; D017312:Toremifene