title: Advanced aggressive fibromatosis: Effective palliation with chemotherapy.
abstract: BACKGROUND
  Aggressive fibromatosis (AF) is a locally invasive proliferative disease. The mainstay of treatment is surgery. Chemotherapy may be considered in inoperable AF following failure of hormonal therapy and/or NSAIDs.
  
  
  METHODS
  We conducted a retrospective search of the prospectively maintained Royal Marsden Hospital Sarcoma Unit database to identify patients with AF treated with chemotherapy between 1987 and 2009.
  
  
  RESULTS
  Thirty-nine patients, thirty one females and eight males, received one or more lines of chemotherapy. The most frequently employed chemotherapy regimens were methotrexate/vinblastine [MTX/VBL] (18) and pegylated liposomal doxorubicin [PLD] (14). MTX/VBL was administered weekly or every two weeks at MTX 50 mg and VBL 10 mg. Treatment duration ranged from three weeks to one year with a median of 4.5 months. Partial response (PR) was observed in 11% of cases, disease stabilisation (SD) in 60% and progressive disease (PD) in 22%. Time to progression ranged from one month to sixteen years. The main toxicities reported were mucositis (4), peripheral neuropathy (3), vomiting (3), and neutropenia (3). PLD was administered at 40-50 mg/m(2) every four weeks, for up to six cycles. PR was achieved in 33% and in the remainder the disease was stable with no progression during treatment. Three (25%) patients have so far progressed after treatment. Symptomatic benefit, especially pain relief, was reported in 86% (12/14) of cases. Main toxicities included palmar plantar erythema (5) and mucositis (4).
  
  
  CONCLUSIONS
  MTX/VBL remains a useful combination but PLD is emerging as a well tolerated and effective systemic therapy in advanced AF.
authors.affiliation: Sarcoma Unit, Royal Marsden Hospital, London, United Kingdom. anastasia.constantinidou@rmh.nhs.uk
authors.forename: Anastasia
authors.initials: A
authors.lastname: Constantinidou
authors.forename: Robin L
authors.initials: RL
authors.lastname: Jones
authors.forename: Michelle
authors.initials: M
authors.lastname: Scurr
authors.forename: Omar
authors.initials: O
authors.lastname: Al-Muderis
authors.forename: Ian
authors.initials: I
authors.lastname: Judson
mesh_terms: D000008:Abdominal Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology; D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D004317:Doxorubicin / Q000008:administration & dosage / Q000031:analogs & derivatives; D005260:Female; D018222:Desmoid Tumors / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D010166:Palliative Care / Q000379:methods*; D011092:Polyethylene Glycols / Q000008:administration & dosage; D011859:Radiography; D012042:Registries / Q000706:statistics & numerical data; D012189:Retrospective Studies; D016896:Treatment Outcome; D014747:Vinblastine / Q000008:administration & dosage; D055815:Young Adult
chemical_list: C506643:liposomal doxorubicin; D011092:Polyethylene Glycols; D014747:Vinblastine; D004317:Doxorubicin; D008727:Methotrexate