Background: Desmoid tumours are soft tissue sarcomas with local aggressive behaviour and a high rate of local recurrence after treatment. Although they do not tend to metastasise systemically, the local aggressiveness can lead to situations in which limb-preserving surgery cannot be performed without severe disability. As isolated limb perfusion (ILP) with TNF and melphalan has proven to be extremely effective in the treatment of soft tissue sarcoma, we studied its potential in locally advanced extremity desmoid tumours. Methods: Prospectively maintained database in a tertiary referral centre. Between 1991 and 2003, 12 ILP procedures were performed in 11 patients for locally advanced desmoid tumours. Local surgical therapy with preservation of limb function was impossible in all patients due to large or multifocal tumours, multiple recurrences or extensive previous treatment. Perfusions were performed with 4-3 mg TNF and 10-13 mg/l limb volume melphalan form leg and arm perfusions, respectively. Results: Overall response rate was 75%: Two complete responses were recorded (17%) and seven patients had a partial response (58%). Amputation could be avoided in all cases. Local control was obtained after 10/12 ILPs and in the other two patients through repeat ILP and systemic chemotherapy, thus leading to an overall local control rate of 100%. Local toxicity was mild and systemic toxicity was absent in all patients. Conclusion: ILP is a very effective treatment option in the multimodality treatment of limb desmoid tumours. It should be considered in patients with aggressive and disabling disease where resection without important functional sacrifice is impossible.

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Keywords Aggressive fibromatosis, Isolated limb perfusion, Melphalan, Soft tissue sarcoma, TNF
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Journal European Journal of Surgical Oncology
Grünhagen, D.J, de Wilt, J.H.W, Verhoef, C, van Geel, A.N, & Eggermont, A.M.M. (2005). TNF-based isolated limb perfusion in unresectable extremity desmoid tumours. European Journal of Surgical Oncology, 31(8), 912–916. doi:10.1016/j.ejso.2005.07.002