Sarcoma Indifferenziato (embrionale) del fegato in età pediatrica L'esperienza del Cruppo Cooperative Italiano per il trattamento dei Sarcomi delle Parti Molli in età pediatrica

Translated title of the contribution: Undifferentiated (embryonal) sarcoma of the liver in childhood the experience of the Italian Cooperative croup on Pédiatrie Soft Tissue Sarcoma

G. Bisogno, G. Cecchetto, P. Dall'Igna, E. Talenti, G. Scarzello, E. Basso, A. Ferrari, S. Dallorso, A. Mancini, F. Massolo, I. Zanetti, M. Carli

Research output: Contribution to journalArticle

Abstract

Introduction The undifferentiated (embryonal) sarcoma of the liver (USL) is a rare mesenchymal tumor that occurs mainly in children benveen 5 and 10 years. Prognosis is reputed poor because radical surgery is difficult and the activity of chemotherapy is not well documented. Methods Ten children with USL have been enrolled in the protocols ran by the Italian Cooperative Group from 1979 to 1995. Treatment was similar to the one used for children with rhabdomyosarcoma and provided a conservative radical surgery at diagnosis or after preoperative chemotherapy and eventually radiotherapy. Results Surgery was radical in 5 patients: at diagnosis (one case) or after chemotherapy. Response to initial chemotherapy was evident in 6 out of 7 évaluable cases. Six patients are alive without evidence of disease 5 to 12 years from diagnosis. One child died for surgical complication and ftvo because of the tumor. One more patient is lost to follow up. Conclusions Our results confirm the crucial role of radical resection of the tumor. Chemotherapy may be administered to reduce the tumor size making the mass resection feasible. Improvement in surgical techniques and administration of aggressive chemotherapy, similar to that used for rhabdomyosarcom, has improved the prognosis for these patients.

Original languageItalian
Pages (from-to)320-327
Number of pages8
JournalRivista Italiana di Pediatria
Volume26
Issue number2
Publication statusPublished - 2000

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Croup
Sarcoma
Drug Therapy
Liver
Neoplasms
Rhabdomyosarcoma
Lost to Follow-Up
Radiotherapy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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Sarcoma Indifferenziato (embrionale) del fegato in età pediatrica L'esperienza del Cruppo Cooperative Italiano per il trattamento dei Sarcomi delle Parti Molli in età pediatrica. / Bisogno, G.; Cecchetto, G.; Dall'Igna, P.; Talenti, E.; Scarzello, G.; Basso, E.; Ferrari, A.; Dallorso, S.; Mancini, A.; Massolo, F.; Zanetti, I.; Carli, M.

In: Rivista Italiana di Pediatria, Vol. 26, No. 2, 2000, p. 320-327.

Research output: Contribution to journalArticle

Bisogno, G. ; Cecchetto, G. ; Dall'Igna, P. ; Talenti, E. ; Scarzello, G. ; Basso, E. ; Ferrari, A. ; Dallorso, S. ; Mancini, A. ; Massolo, F. ; Zanetti, I. ; Carli, M. / Sarcoma Indifferenziato (embrionale) del fegato in età pediatrica L'esperienza del Cruppo Cooperative Italiano per il trattamento dei Sarcomi delle Parti Molli in età pediatrica. In: Rivista Italiana di Pediatria. 2000 ; Vol. 26, No. 2. pp. 320-327.
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abstract = "Introduction The undifferentiated (embryonal) sarcoma of the liver (USL) is a rare mesenchymal tumor that occurs mainly in children benveen 5 and 10 years. Prognosis is reputed poor because radical surgery is difficult and the activity of chemotherapy is not well documented. Methods Ten children with USL have been enrolled in the protocols ran by the Italian Cooperative Group from 1979 to 1995. Treatment was similar to the one used for children with rhabdomyosarcoma and provided a conservative radical surgery at diagnosis or after preoperative chemotherapy and eventually radiotherapy. Results Surgery was radical in 5 patients: at diagnosis (one case) or after chemotherapy. Response to initial chemotherapy was evident in 6 out of 7 {\'e}valuable cases. Six patients are alive without evidence of disease 5 to 12 years from diagnosis. One child died for surgical complication and ftvo because of the tumor. One more patient is lost to follow up. Conclusions Our results confirm the crucial role of radical resection of the tumor. Chemotherapy may be administered to reduce the tumor size making the mass resection feasible. Improvement in surgical techniques and administration of aggressive chemotherapy, similar to that used for rhabdomyosarcom, has improved the prognosis for these patients.",
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AU - Bisogno, G.

AU - Cecchetto, G.

AU - Dall'Igna, P.

AU - Talenti, E.

AU - Scarzello, G.

AU - Basso, E.

AU - Ferrari, A.

AU - Dallorso, S.

AU - Mancini, A.

AU - Massolo, F.

AU - Zanetti, I.

AU - Carli, M.

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