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Fadi Farhat
 Updated on January 28th, 1998.

"Desmoplastic Small Round Cell Tumors: Results of a Four-Drug Chemotherapy Regimen in Five Adult Patients"


by Fadi Farhat, et al.

This was a study from Nov. 1991 to Jan. 1995 which consisted of five patients (3 women and 2 men) with DSRCT who received a uniform cisplatin based chemotherapy as part of the first line/salvage treatment at the Institut Gustave Roussy. The median age at diagnosis was 22 years. The clinical presentation in four patients was intra-abdominal, and one patient had para-testicular primary. Three patients had suboptimal debulking, one patient had a biopsy, and the patient with para-testicular disease underwent a left orchiectomy at diagnosis and was referred at relapse.

The treatment regimens consisted of PAVEP (cyclophosphamide, etoposide, doxorubicin, and cisplatin) every four weeks. The PEVEP substituted doxorubicin for epirubicin.

The PAVEP regimen was given to the four patients with intra-abdominal presentation after surgery. The patient with para-testicular disease received the PEVEP regimen 6 months after initial orchiectomy due to bone-lung metastases along with mediastinal and para-aortic lymph node enlargement was noted. All four patients with intra-abdominal disease experienced stability ranging from 4 to 9 months. Only one complete response was observed in patient five. After four cycles of the PEVEP, two courses of high dose carboplatin, etoposide, and ifosfamide, with stem cell support was given as consolidation. This patient is still alive with no signs of recurrence 32 months after diagnosis.

The optimal treatment of DSRCT is not known. Debulking is necessary for symptomatic relief, yet the impact on survival is unclear. Inguinal orchiectomy should be done in all primary testicular tumors. Radio-therapy used for palliation was found not to have any influence in the course of the disease as a curative dose could not be given. This is due to the low tolerance of abdominal organs and the extent of intra-abdominal disease. Yet one patient has had complete remission lasting 42 months and was treated with abdominal radiation associated with 5-FU. In summary of the literature, which included 60 patients who were treated with chemotherapy with and without radiation therapy, objective responses were noted in 17 patients, 8 of whom had complete response. three of the 8 developed recurrences. The other 5 are in complete remission after 4, 12, 24 42, and 48 months. The regimens associated with complete responses were essentially those using doxorubicin, cyclophophamide, vincristine, and cisplatin.


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