BACKGROUND: Desmoid tumours are rare neoplasms characterised by clonal proliferation of myofibroblasts that do not metastasise, but often exhibit an infiltrative pattern and functional impairment. When desmoids arise in the intestinal mesentery, surgical resection is seldom possible without life-altering loss of intestinal function. METHODS: Retrospective review of the clinical management of 52 consecutive patients treated for desmoids of the intestinal mesentery from January 2001 to August 2006. A multidisciplinary treatment plan was developed based on primary disease extent, tumour behaviour and resectability. Patients with stable but unresectable disease were observed without treatment. Patients with resectable disease underwent surgery, and patients with unresectable progressing disease received chemotherapy, most commonly liposomal doxorubicin, followed by surgery if chemotherapy rendered the disease resectable. RESULTS: At a median follow-up of 50.0 months (range 4.6-212), 50 patients (96%) have either no recurrence or radiographically stable disease. No patient requires total parenteral nutrition. CONCLUSION: These data indicate that the extent of disease; tumour behaviour and resectability are the important factors when defining a treatment plan for mesenteric desmoid tumours. A multidisciplinary approach of surgery combined with chemotherapy is an effective and function-sparing strategy for managing this disease.
BACKGROUND:Desmoid tumours are rare neoplasms characterised by clonal proliferation of myofibroblasts that do not metastasise, but often exhibit an infiltrative pattern and functional impairment. When desmoids arise in the intestinal mesentery, surgical resection is seldom possible without life-altering loss of intestinal function. METHODS: Retrospective review of the clinical management of 52 consecutive patients treated for desmoids of the intestinal mesentery from January 2001 to August 2006. A multidisciplinary treatment plan was developed based on primary disease extent, tumour behaviour and resectability. Patients with stable but unresectable disease were observed without treatment. Patients with resectable disease underwent surgery, and patients with unresectable progressing disease received chemotherapy, most commonly liposomal doxorubicin, followed by surgery if chemotherapy rendered the disease resectable. RESULTS: At a median follow-up of 50.0 months (range 4.6-212), 50 patients (96%) have either no recurrence or radiographically stable disease. No patient requires total parenteral nutrition. CONCLUSION: These data indicate that the extent of disease; tumour behaviour and resectability are the important factors when defining a treatment plan for mesenteric desmoid tumours. A multidisciplinary approach of surgery combined with chemotherapy is an effective and function-sparing strategy for managing this disease.
Authors: Adelaide M Carothers; Hira Rizvi; Rian M Hasson; Yvonne I Heit; Jennifer S Davids; Monica M Bertagnolli; Nancy L Cho Journal: Cancer Res Date: 2011-11-17 Impact factor: 12.701