Desmoplastic small round cell tumour in children and adolescents
| العنوان: | Desmoplastic small round cell tumour in children and adolescents |
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| المؤلفون: | Vito Ninfo, Jelena Roganovich, Luca Cordero di Montezemolo, Gianni Bisogno, Alberto Donfrancesco, Guido Sotti, Maurizio Mascarin, Modesto Carli |
| المصدر: | Università degli Studi di Padova-IRIS |
| بيانات النشر: | Wiley, 2000. |
| سنة النشر: | 2000 |
| مصطلحات موضوعية: | Male, Cancer Research, medicine.medical_specialty, Vincristine, Adolescent, medicine.medical_treatment, Disease-Free Survival, Testicular Neoplasms, Antineoplastic Combined Chemotherapy Protocols, medicine, Humans, Ifosfamide, Child, Antineoplastic Agents, Alkylating, Salvage Therapy, Chemotherapy, Antibiotics, Antineoplastic, business.industry, Soft tissue sarcoma, Remission Induction, Prognosis, medicine.disease, Antineoplastic Agents, Phytogenic, Combined Modality Therapy, Chemotherapy regimen, Surgery, Survival Rate, Radiation therapy, Oncology, Abdominal Neoplasms, Sarcoma, Small Cell, Pediatrics, Perinatology and Child Health, Dactinomycin, Sarcoma, Neoplasm Recurrence, Local, business, medicine.drug, Epirubicin |
| الوصف: | Background Desmoplastic small round cell tumour (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, macro-scopically 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. Med. Pediatr. Oncol. 34:338–342, 2000. © 2000 Wiley-Liss, Inc. |
| تدمد: | 1096-911X 0098-1532 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::35acd221d8513c087ae25a917bbef6a9 https://doi.org/10.1002/(sici)1096-911x(200005)34:5<338::aid-mpo4>3.0.co;2-0 |
| حقوق: | CLOSED |
| رقم الأكسشن: | edsair.doi.dedup.....35acd221d8513c087ae25a917bbef6a9 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 1096911X 00981532 |
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