title: Use of doxorubicin and dacarbazine for the management of unresectable intra-abdominal desmoid tumors in Gardner's syndrome.
abstract: OBJECTIVE
  The aim of this study was to describe the natural history and management of surgically unresectable intra-abdominal desmoid tumors in two patients with Gardner's syndrome from two unrelated families, where each had failed on conventional therapy.
  
  
  METHODS
  Two patients with Gardner's syndrome were placed on a chemotherapy regimen which included doxorubicin (90 mg/m2) and dacarbazine (900 mg/m2) in divided doses over four days of continuous infusion. Their progress on chemotherapy was assessed by abdominal computerized tomography and laparoscopy.
  
  
  RESULTS
  The computerized abdominal tomography scans proved difficult to interpret because of adhesions and matted small bowel resulting from the patients original colectomies. These findings made it difficult to differentiate postoperative changes from residual desmoid tumor. Second-look laparotomy in such patients was contraindicated as this may predispose to further desmoid production. Laparoscopy disclosed a complete response to this chemotherapy. Nevertheless, we had an iatrogenic small bowel perforation in one of these patients. Each patient showed a complete response to chemotherapy.
  
  
  CONCLUSIONS
  Surgical resection remains the first-line treatment of intraabdominal desmoid tumors. However, doxorubicin/dacarbazine chemotherapy on a clinical trial basis may be indicated in patients whose intra-abdominal desmoid is unresectable, or who have failed to respond to treatment with hormones (tamoxifen, Toremifene), steroids (prednisone), and nonsteroidal anti-inflammatory agents (Clinoril; Merck & Co., Inc., West Point, PA).
authors.affiliation: Department of Preventive Medicine and Public Health, Creighton University School of Medicine, Omaha, Nebraska.
authors.forename: H T
authors.initials: HT
authors.lastname: Lynch
authors.forename: R
authors.initials: R
authors.lastname: Fitzgibbons
authors.forename: S
authors.initials: S
authors.lastname: Chong
authors.forename: J
authors.initials: J
authors.lastname: Cavalieri
authors.forename: J
authors.initials: J
authors.lastname: Lynch
authors.forename: F
authors.initials: F
authors.lastname: Wallace
authors.forename: S
authors.initials: S
authors.lastname: Patel
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003606:Dacarbazine / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D018221:Fibromatosis, Abdominal / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy*; D005736:Gardner Syndrome / Q000150:complications* / Q000235:genetics; D006801:Humans; D010535:Laparoscopy; D008297:Male; D010375:Pedigree; D014057:Tomography, X-Ray Computed
chemical_list: D003606:Dacarbazine; D004317:Doxorubicin