title: Desmoplastic small round cell tumors: results of a four-drug chemotherapy regimen in five adult patients.
abstract: BACKGROUND
  Desmoplastic small round-cell tumor has been identified as a neoplasm with multidirectional immunohistochemical, and molecular features of this tumor sets it apart as a pathologic entity. The optimal treatment remains to be determined.
  
  
  METHODS
  Five adult patients were treated according to a uniform first-line chemotherapy program including cisplatin, etoposide, cyclophosphamide, and either doxorubicin or epirubicin. Chemotherapy was delivered after initial surgery in the four patients with intra-abdominal presentation, and at relapse in the fifth patient who had a paratesticular primary tumor.
  
  
  RESULTS
  All 4 patients with intra-abdominal disease experienced stability lasting from 4 to 9 months. Only one objective persistent complete response was observed; this was in the patient with a paratesticular primary. No salvage treatment was active in the other four patients who died of progressive disease.
  
  
  CONCLUSIONS
  Our experience to a certain degree of chemosensitivity for desmoplastic small round-cell tumors. Despite aggressive treatments, survival rates remain disappointing. Other therapeutic modalities are needed to improve these results.
authors.affiliation: Department of Medicine, Institut Gustave Roussy, Villejuif, France.
authors.forename: F
authors.initials: F
authors.lastname: Farhat
authors.forename: S
authors.initials: S
authors.lastname: Culine
authors.forename: C
authors.initials: C
authors.lastname: Lhommé
authors.forename: P
authors.initials: P
authors.lastname: Duvillard
authors.forename: P
authors.initials: P
authors.lastname: Soulié
authors.forename: G
authors.initials: G
authors.lastname: Michel
authors.forename: M J
authors.initials: MJ
authors.lastname: Terrier-Lacombe
authors.forename: C
authors.initials: C
authors.lastname: Théodore
authors.forename: M
authors.initials: M
authors.lastname: Schreinerova
authors.forename: J P
authors.initials: JP
authors.lastname: Droz
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use*; D018288:Carcinoma, Small Cell / Q000188:drug therapy*; D002945:Cisplatin / Q000008:administration & dosage; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D008297:Male; D010534:Peritoneal Neoplasms / Q000188:drug therapy*; D016879:Salvage Therapy; D016896:Treatment Outcome
chemical_list: D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D002945:Cisplatin