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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