title: Isolated limb perfusion using tumour necrosis factor α and melphalan in patients with advanced aggressive fibromatosis.
abstract: BACKGROUND
  Aggressive fibromatoses (desmoid tumours) may be locally aggressive, but do not metastasize. Although a conservative approach is advocated for most patients, pain and functional impairment are indications for active treatment. Tumour necrosis factor (TNF) α and melphalan-based isolated limb perfusion (TM-ILP) is a limb-saving treatment modality for soft tissue tumours. This study reports the results of TM-ILP treatment in patients with aggressive fibromatosis.
  
  
  METHODS
  Institutional databases of three European centres were searched. All patients who received TM-ILP treatment for aggressive fibromatosis between 1990 and 2012 were included. Before therapy, the patients were discussed at multidisciplinary tumour board meetings.
  
  
  RESULTS
  Twenty-five patients received 28 TM-ILP treatments. The median age of patients was 28 (i.q.r. 19-34) years and median hospital stay was 8 (7-12) days. Median follow-up was 84 (34-114) months. A complete response was achieved after two TM-ILP treatments, and a partial response after 17 treatments in 16 patients. Stable disease was reported after eight treatments in seven patients, including a patient with stable disease after the first treatment and progression after the second TM-ILP. Toxicity was modest after most treatments; Wieberdink grade IV (extensive epidermolysis, and threatening or manifest compartment syndrome) was seen after two TM-ILP treatments. Systemic leakage was reported after one treatment, but did not lead to systemic toxicity. Functional outcome was good; 16 patients had no physical limitations, and six patients had some limitations but did not need medical aids. Amputation was prevented in all but three patients.
  
  
  CONCLUSIONS
  TNF-α-based ILP is effective in patients with aggressive fibromatosis.
authors.affiliation: Department of Surgical Oncology, Erasmus Medical Centre Cancer Institute, Rotterdam, The Netherlands.
authors.forename: D L M
authors.initials: DL
authors.lastname: van Broekhoven
authors.forename: J P
authors.initials: JP
authors.lastname: Deroose
authors.forename: S
authors.initials: S
authors.lastname: Bonvalot
authors.forename: A
authors.initials: A
authors.lastname: Gronchi
authors.forename: D J
authors.initials: DJ
authors.lastname: Grünhagen
authors.forename: A M M
authors.initials: AM
authors.lastname: Eggermont
authors.forename: C
authors.initials: C
authors.lastname: Verhoef
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001132:Arm; D010478:Chemotherapy, Cancer, Regional Perfusion / Q000379:methods; D005260:Female; D018221:Fibromatosis, Abdominal / Q000188:drug therapy*; D006801:Humans; D007866:Leg; D008297:Male; D008558:Melphalan / Q000008:administration & dosage / Q000009:adverse effects; D009364:Neoplasm Recurrence, Local / Q000628:therapy; D011446:Prospective Studies; D016896:Treatment Outcome; D014409:Tumor Necrosis Factor-alpha / Q000008:administration & dosage / Q000009:adverse effects; D055815:Young Adult
chemical_list: D014409:Tumor Necrosis Factor-alpha; D008558:Melphalan