Abstract
Background. Desmoplastic small round cell rumour (DSRCT) is a rare highly aggressive neoplasm, and clinical studies are scarce. Procedure. We report six cases of children and adolescents (median age 14 years, range 6.9- 17.5) with DSRCT (5 abdominal, 1 paratesticular) registered by the Italian Cooperative Group (ICG) for soft tissue sarcoma over a 9-year period. Patients received a multidisciplinary treatment, including aggressive initial or delayed surgery and radiotherapy. Chemotherapy regimen was based on the use of ifosfamide, vincristine, dactinomycin, and a few doses of antharacyclines (doxorubicin or epirubicin). Results. Complete surgical resection was possible only for the paratesticular tumour. Among the patients with abdominal lesions, macroscopically radical excision was possible in only one case. All patients received multidrug chemotherapy, and tumour reduction was obtained in the 4 evaluable patients. No relapses were evident in the irradiated fields in the 4 patients who received radiotherapy. Two patients remained progression-free 22 and 63 months after diagnosis, one is in third complete remission, whereas three died 10-25 months after diagnosis. Conclusions. DSRCT is a chemosensitive tumour, but survival rates remain disappointing despite aggressive multimodality therapy. Our results support surgical tumour removal and radiotherapy to achieve local control. Our experience and a review of the literature suggest that patients with localised abdominal tumours or a paratesticular primary may have a better prognosis. (C) 2000 Wiley-Liss, Inc.
| Original language | English |
|---|---|
| Pages (from-to) | 338-342 |
| Number of pages | 5 |
| Journal | Medical and Pediatric Oncology |
| Volume | 34 |
| Issue number | 5 |
| DOIs | |
| Publication status | Published - May 2000 |
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Keywords
- Childhood cancer
- Combined modality care
- Desmoplastic small round cell tumour
- Sarcoma
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Oncology
- Cancer Research
Cite this
Desmoplastic small round cell tumor in children and adolescents. / Bisogno, Gianni; Roganovich, Jelena; Sotti, Guido; Ninfo, Vito; Di Montezemolo, Luca Cordero; Donfrancesco, Alberto; Mascarin, Maurizio; Carli, Modesto.
In: Medical and Pediatric Oncology, Vol. 34, No. 5, 05.2000, p. 338-342.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Desmoplastic small round cell tumor in children and adolescents
AU - Bisogno, Gianni
AU - Roganovich, Jelena
AU - Sotti, Guido
AU - Ninfo, Vito
AU - Di Montezemolo, Luca Cordero
AU - Donfrancesco, Alberto
AU - Mascarin, Maurizio
AU - Carli, Modesto
PY - 2000/5
Y1 - 2000/5
N2 - Background. Desmoplastic small round cell rumour (DSRCT) is a rare highly aggressive neoplasm, and clinical studies are scarce. Procedure. We report six cases of children and adolescents (median age 14 years, range 6.9- 17.5) with DSRCT (5 abdominal, 1 paratesticular) registered by the Italian Cooperative Group (ICG) for soft tissue sarcoma over a 9-year period. Patients received a multidisciplinary treatment, including aggressive initial or delayed surgery and radiotherapy. Chemotherapy regimen was based on the use of ifosfamide, vincristine, dactinomycin, and a few doses of antharacyclines (doxorubicin or epirubicin). Results. Complete surgical resection was possible only for the paratesticular tumour. Among the patients with abdominal lesions, macroscopically radical excision was possible in only one case. All patients received multidrug chemotherapy, and tumour reduction was obtained in the 4 evaluable patients. No relapses were evident in the irradiated fields in the 4 patients who received radiotherapy. Two patients remained progression-free 22 and 63 months after diagnosis, one is in third complete remission, whereas three died 10-25 months after diagnosis. Conclusions. DSRCT is a chemosensitive tumour, but survival rates remain disappointing despite aggressive multimodality therapy. Our results support surgical tumour removal and radiotherapy to achieve local control. Our experience and a review of the literature suggest that patients with localised abdominal tumours or a paratesticular primary may have a better prognosis. (C) 2000 Wiley-Liss, Inc.
AB - Background. Desmoplastic small round cell rumour (DSRCT) is a rare highly aggressive neoplasm, and clinical studies are scarce. Procedure. We report six cases of children and adolescents (median age 14 years, range 6.9- 17.5) with DSRCT (5 abdominal, 1 paratesticular) registered by the Italian Cooperative Group (ICG) for soft tissue sarcoma over a 9-year period. Patients received a multidisciplinary treatment, including aggressive initial or delayed surgery and radiotherapy. Chemotherapy regimen was based on the use of ifosfamide, vincristine, dactinomycin, and a few doses of antharacyclines (doxorubicin or epirubicin). Results. Complete surgical resection was possible only for the paratesticular tumour. Among the patients with abdominal lesions, macroscopically radical excision was possible in only one case. All patients received multidrug chemotherapy, and tumour reduction was obtained in the 4 evaluable patients. No relapses were evident in the irradiated fields in the 4 patients who received radiotherapy. Two patients remained progression-free 22 and 63 months after diagnosis, one is in third complete remission, whereas three died 10-25 months after diagnosis. Conclusions. DSRCT is a chemosensitive tumour, but survival rates remain disappointing despite aggressive multimodality therapy. Our results support surgical tumour removal and radiotherapy to achieve local control. Our experience and a review of the literature suggest that patients with localised abdominal tumours or a paratesticular primary may have a better prognosis. (C) 2000 Wiley-Liss, Inc.
KW - Childhood cancer
KW - Combined modality care
KW - Desmoplastic small round cell tumour
KW - Sarcoma
UR - http://www.scopus.com/inward/record.url?scp=0034093381&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0034093381&partnerID=8YFLogxK
U2 - 10.1002/(SICI)1096-911X(200005)34:5<338::AID-MPO4>3.0.CO;2-0
DO - 10.1002/(SICI)1096-911X(200005)34:5<338::AID-MPO4>3.0.CO;2-0
M3 - Article
C2 - 10797355
AN - SCOPUS:0034093381
VL - 34
SP - 338
EP - 342
JO - Pediatric Blood and Cancer
JF - Pediatric Blood and Cancer
SN - 1545-5009
IS - 5
ER -