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Surgical view of the treatment of patients with hepatoblastoma. Results from the First Prospective Trial of the International Society of Pediatric Oncology Liver Tumor Study Group (SIOPEL-1).
Cancer 2002: 94 (4) s.1111-1120, il., tab., bibliogr. 20 poz.
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Background. Surgical resection is the cornerstone of treatment for patients with hepatoblastoma (HB). The Society of Pediatric Oncology Liver Tumor Study Group launched its first prospective trial (SIOPEL-1) with the intention to treat all patients with preoperative chemotherapy and delayed surgical resection. The objective of this article was to assess the assumed surgical advantages of primary chemotherapy. Methods. Between 1990 and 1994, 154 patients age 16 years with HB were registered on SIOPEL-1. The pretreatment extent of disease was assessed, and, after undergoing biopsy, patients were treated with cisplatin 80 mg/mý intravenously over 24 hours and doxorubicin 60 mg/mý intravenously over 48 hours by continuous infusion (PLADO). Generally, tumors were resected after four of a total of six courses of PLADO. Results. One hundred twenty eight patients underwent surgical resection (13 patients underwent primary surgery, and 115 patients underwent delayed surgery after PLADO). A pretreatment surgical biopsy was performed in 96 of 128 patients (75 p.c.). Biopsy complications occurred in 7 of 96 patients (7 p.c.). Twenty-two patients showed pulmonary metastases at the time of diagnosis, and 7 patients underwent thoracotomy. Operative morbidity and mortality were 18 p.c. and 5 p.c., respectively. Complete macroscopic surgical resection was achieved in 106 patients (92 p.c.), including 6 patients who underwent orthotopic liver transplantation. The actuarial 5-year event free survival (EFS) rate for all 154 patients in the study was 66 p.c., and the overall survival (OS) rate was (75 p.c.)...
Hepatocellular carcinoma in children - treatment perspectives on the basis of international SIOPEL-1 trial experience.
Ann. Diagn. Paediatr. Pathol. 2002: 6 (1/2) s.27-33, il., tab., bibliogr. 43 poz. - 2 Sympozjum pt. Postępy w diagnostyce molekularnej i leczeniu nowotworów złośliwych na podłożu genetycznym w pediatrii Zegrze-Południe 08-09.05. 2002
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dzieci 2-5 r.ż.
dzieci 6-12 r.ż.
dzieci 13-18 r.ż.
Introduction: Hepatocellular carcinoma (HCC) is the second most common malignant liver neoplasms (after hepatoblastoma) in the pediatric population. Objectives: To present information on outcome and prognosis of pediatric HCC and to discuses eventual treatment strategies. Material: Forty patients diagnosed with hepatocellular carcinoma (HCC) were registered in the SIOPEL-1 study from 1990 to 1994 (39 were evaluable). Metastases at diagnosis occurred in 31 p.c. children. Multifocal tumors prevailed (56 p.c.). 37 patients received preoperative chemotherapy (PLADO - cisplatin and doxorubicin). Results: Partial response was observed in 18/37 cases (49 p.c.). Complete tumor resection was achieved in 14 patients (36 p.c.) (including 2 liver transplantations). Twenty cases (51 p.c.) never became operable. Overall survival (OS) at 5 yrs is 28 p.c., while event free survival (EFS) in 17 p.c. The overall poor treatment results of HCC patients were mainly due to advanced tumor stage and low rate of complete excision. In multivariate analysis metastates and high PRETEXT grouping were identified as adverse prognostic factors. Despite fairly high response rate to preoperative chemotherapy over 50 p.c. of tumors remained inoperable. Different therapeutic options in HCC, including: surgery, transplantation, tumor embolization and chemoembolization, ethanol injection, immuno- and hormonotherapy, as well as antiangiogenic therapy, are presented and discussed. Conclusions: 1. Generally poor survival in pediatric hepatocellular carcinoma at modern standards of care...
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