A systematic review of active treatment options in patients with desmoid tumours

X. Yao, T. Corbett, A. Gupta, R.A. Kandel, S. Verma, J. Werier, M. Ghert



We conducted a systematic review to determine the optimal treatment options in patients with desmoid tumours who have declined observational management.


A search was conducted of the medline and embase databases (1990 to September 2012), the Cochrane Library, and relevant guideline Web sites and conference materials.


One systematic review and forty-six studies met the preplanned study selection criteria; data from twenty-eight articles were extracted and analyzed. For local control, three studies reported a statistically significant difference in favour of surgery plus radiotherapy (rt) compared with surgery alone, and one study did not; two studies reported the lack of a statistical difference between surgery plus rt and rt alone in maintaining local control. Multivariate risk factors for local recurrence included positive surgical margins and young patient age. Single-agent imatinib led to a progression-free survival rate of 55% at 2 years and 58% at 3 years. Methotrexate plus vinblastine led to a progression-free survival rate of 67% at 10 years. Significant toxicities were reported for all treatment modalities, including surgical morbidity, and rt- and chemotherapy-related toxicities.


In patients who have declined observational management, the local control rate was higher with surgery plus rt than with surgery alone. However, the additional rt-related complications should be considered in treatment decision-making. Surgery, rt, and systemic therapy are all reasonable treatment options for patients with desmoid tumours.


Desmoid tumours; systematic review; treatments

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DOI: http://dx.doi.org/10.3747/co.21.1995

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ISSN: 1198-0052 (Print) ISSN: 1718-7729 (Online)