Desmoplastic small round cell tumors: results of a four-drug chemotherapy regimen in five adult patients.
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.
Farhat
F
F
Department of Medicine, Institut Gustave Roussy, Villejuif, France.
Culine
S
S
Lhommé
C
C
Duvillard
P
P
Soulié
P
P
Michel
G
G
Terrier-Lacombe
M J
MJ
Théodore
C
C
Schreinerova
M
M
Droz
J P
JP
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
D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D002945:Cisplatin