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Titolo:
Desmoplastic small round cell tumour in children and adolescents
Autore:
Bisogno, G; Roganovich, J; Sotti, G; Ninfo, V; di Montezemolo, LC; Donfrancesco, A; Mascarin, M; Carli, M;
Indirizzi:
Univ Padua, Dipartimento Pediat, Div Oncoematol, I-35128 Padua, Italy UnivPadua Padua Italy I-35128 iat, Div Oncoematol, I-35128 Padua, Italy Div Radiotherapy, Padua, Italy Div Radiotherapy Padua ItalyDiv Radiotherapy, Padua, Italy Univ Padua, Inst Pathol, Padua, Italy Univ Padua Padua ItalyUniv Padua, Inst Pathol, Padua, Italy Univ Turin, Paediat Oncol Div, Turin, Italy Univ Turin Turin ItalyUniv Turin, Paediat Oncol Div, Turin, Italy Osped Bambin Gesu, Div Oncol, Rome, Italy Osped Bambin Gesu Rome ItalyOsped Bambin Gesu, Div Oncol, Rome, Italy CRO, Div Radiotherapy, Aviano, Italy CRO Aviano ItalyCRO, Div Radiotherapy, Aviano, Italy
Titolo Testata:
MEDICAL AND PEDIATRIC ONCOLOGY
fascicolo: 5, volume: 34, anno: 2000,
pagine: 338 - 342
SICI:
0098-1532(200005)34:5<338:DSRCTI>2.0.ZU;2-B
Fonte:
ISI
Lingua:
ENG
Soggetto:
AGGRESSIVE MULTIMODALITY THERAPY;
Keywords:
desmoplastic small round cell tumour; sarcoma; childhood cancer; combined modality care;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
18
Recensione:
Indirizzi per estratti:
Indirizzo: Bisogno, G Univ Padua, Dipartimento Pediat, Div Oncoematol, Via Giustiniani 3, I-35128 Padua, Italy Univ Padua Via Giustiniani 3 Padua Italy I-35128 Padua, Italy
Citazione:
G. Bisogno et al., "Desmoplastic small round cell tumour in children and adolescents", MED PED ONC, 34(5), 2000, pp. 338-342

Abstract

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, macroscopically radical excision was possible in only one case. All patients received multidrug chemotherapy, and tumour reduction was obtainedin 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 tumoursor a paratesticular primary may have a better prognosis. (C) 2000 Wiley-Liss, Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 04/12/20 alle ore 16:27:32