Key result
A four-drug chemotherapy regimen resulted in disease stability for 4 to 9 months in 4 of 5 adult patients, though 4 ultimately died of progressive disease.
Why the study?
Does a four-drug chemotherapy regimen improve outcomes in adult patients with desmoplastic small round cell tumors?
Case Report (n=5)
Does a four-drug chemotherapy regimen improve outcomes in adult patients with desmoplastic small round cell tumors?
A four-drug chemotherapy regimen showed limited efficacy in intra-abdominal desmoplastic small round cell tumors, with poor overall survival despite aggressive treatment.
Four-drug regimen offers transient stability but poor survival in adult DSRCT; leaves open need for novel therapies in prospective trials.
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.
No takes yet. Share an insight, caveat, or question.
Farhat et al. (1996) conducted a case report in Desmoplastic small round cell tumors (n=5). Four-drug chemotherapy regimen (cisplatin, etoposide, cyclophosphamide, and doxorubicin or epirubicin) was evaluated on Treatment response and survival. A four-drug chemotherapy regimen resulted in disease stability for 4 to 9 months in 4 of 5 adult patients, though 4 ultimately died of progressive disease.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: