OBJECTIVE: To determine the efficacy of chemotherapy for inoperable desmoid tumours associated with familial adenomatous polyposis. DESIGN: A review of three cases of unresectable desmoid tumours and of the literature on the subject. SETTING: The Steven Atanas Stavro Polyposis Registry at Mount Sinai Hospital in Toronto. PATIENTS: Three patients with symptomatic, unresectable desmoid tumours associated with familial adenomatous polyposis and unresponsive to conventional hormone therapy. INTERVENTION: A chemotherapy regimen of seven cycles of doxorubicin (dose ranging from 60 to 90 mg/m2) and dacarbazine (1000 mg/m2), followed by carboplatin (400 mg/m2) and dacarbazine. OUTCOME MEASURES: Clinical improvement and tumour regression demonstrated by computed tomography. RESULTS: In each of the three cases significant tumour regression was seen clinically and radiologically. CONCLUSIONS: Cytotoxic chemotherapy is an effective treatment for desmoid tumours associated with familial adenomatous polyposis. The chemotherapy should be started early in cases of symptomatic desmoid tumour unresponsive to conventional medical therapy.
OBJECTIVE: To determine the efficacy of chemotherapy for inoperable desmoid tumours associated with familial adenomatous polyposis. DESIGN: A review of three cases of unresectable desmoid tumours and of the literature on the subject. SETTING: The Steven Atanas Stavro Polyposis Registry at Mount Sinai Hospital in Toronto. PATIENTS: Three patients with symptomatic, unresectable desmoid tumours associated with familial adenomatous polyposis and unresponsive to conventional hormone therapy. INTERVENTION: A chemotherapy regimen of seven cycles of doxorubicin (dose ranging from 60 to 90 mg/m2) and dacarbazine (1000 mg/m2), followed by carboplatin (400 mg/m2) and dacarbazine. OUTCOME MEASURES: Clinical improvement and tumour regression demonstrated by computed tomography. RESULTS: In each of the three cases significant tumour regression was seen clinically and radiologically. CONCLUSIONS:Cytotoxic chemotherapy is an effective treatment for desmoid tumours associated with familial adenomatous polyposis. The chemotherapy should be started early in cases of symptomatic desmoid tumour unresponsive to conventional medical therapy.