title: Response of progressive fibromatosis to therapy with liposomal doxorubicin.
abstract: BACKGROUND
  Patients with fibromatosis not amenable to surgery may suffer from high morbidity. Various chemotherapeutic regimens have been tried in these patients with limited success. Here, we report on the successful use of pegylated liposomal doxorubicin in the treatment of 4 patients with unresectable fibromatosis in unfavorable localizations.
  
  
  METHODS
  3 children and 1 adult with progressive fibromatosis were treated with 3-weekly cycles of chemotherapy with liposomal doxorubicin (dose range 20-50 mg/m2 per day every 21 days). Tumors were located at the nasal cavity, fossa infratemporalis, oral cavity, abdomen, and fossa supraclavicularis and were unresectable. 3 of the 4 patients had been heavily pretreated with various chemotherapeutic agents. Objective tumor response was monitored by magnetic resonance imaging and possible cardiotoxicity by echocardiography at regular intervals.
  
  
  RESULTS
  A tumor response was obtained in all 4 patients. All patients showed normal cardiac function after completion of chemotherapy as evaluated by left ventricular shortening fraction. Severe neutropenia was not observed.
  
  
  CONCLUSIONS
  Pegylated liposomal doxorubicin is a therapeutic option in patients with progressive unresectable fibromatosis in unfavorable localizations.
authors.affiliation: Division of Pediatric Hematology/Oncology, Department of Pediatrics and Adolescent Medicine, University of Freiburg, Germany.
authors.forename: G
authors.initials: G
authors.lastname: Wehl
authors.forename: J
authors.initials: J
authors.lastname: Rossler
authors.forename: J E
authors.initials: JE
authors.lastname: Otten
authors.forename: N
authors.initials: N
authors.lastname: Boehm
authors.forename: M
authors.initials: M
authors.lastname: Uhl
authors.forename: U
authors.initials: U
authors.lastname: Kontny
authors.forename: C
authors.initials: C
authors.lastname: Niemeyer
mesh_terms: D000293:Adolescent; D000328:Adult; D000970:Antineoplastic Agents / Q000008:administration & dosage*; D002675:Child, Preschool; D018450:Disease Progression; D004317:Doxorubicin / Q000008:administration & dosage*; D005153:Facial Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000628:therapy; D005260:Female; D018221:Fibromatosis, Abdominal / Q000175:diagnosis / Q000188:drug therapy* / Q000628:therapy; D018222:Desmoid Tumors / Q000175:diagnosis / Q000188:drug therapy* / Q000628:therapy; D006801:Humans; D008297:Male; D009062:Mouth Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000628:therapy; D009669:Nose Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000628:therapy; D017211:Treatment Failure; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D004317:Doxorubicin