BACKGROUND: Hepatoblastoma (HB) is the most common liver tumor of childhood, and comprises approximately 1% of all pediatric malignancies. Although recent data from multicenter trials of GPOH, SIOP, CCG and POG indicate a remarkable improvement of therapy results, the prognosis of advanced or recurrent HB is still not satisfying. PATIENTS AND METHODS: During 1989 and 1997, the German Cooperative Pediatric Liver Tumor Studies HB 89 and HB 94 registered 141 patients with HB, who were treated according to the study protocols. These patients received standard chemotherapy with ifosfamide, cisplatin and doxorubicin (IPA) pre-operatively and/or post-operatively. Fourteen children with recurrent or advanced HB were additionally treated with carboplatin and etoposide (CARBO/VP 16), the reason being observations of drug resistance in children with HB after four or more courses of IPA-therapy in the HB 89 study. The clinical data and course of these patients were evaluated to investigate the efficiency of CARBO/VP 16 chemotherapy and for analyzing the role of surgery. RESULTS: Mean follow-up for survivors was 4.3 years (range 13 months-8 years). Tumor resection was attempted in 13 children but, in only 3 cases, was a complete tumor resection achieved in one operation. There was no perioperative death, and 7 of the patients (50%) are in remission. Two patients underwent adjuvant chemotherapy with CARBO/VP 16 for advanced HB at first operation: all are alive and well. Five patients with local relapse and/or distant metastases responded partially to CARBO/VP 16 therapy, and a complete remission was achieved in one patient. In five patients, progressive disease was observed during therapy with CARBO/VP 16. One patient, stable while on chemotherapy, had a successful resection. Acute toxicity of chemotherapy was observed in 7 patients (50%). CONCLUSION: An aggressive approach using IPA and CARBO/VP 16 chemotherapy and highly developed surgical techniques may improve the prognosis of advanced or recurrent HBs.

Antineoplastic Combined Chemotherapy Protocols↗

PubMedDate not supplied

[Therapy of malignant liver tumors in childhood. An intermediate report of the HB-89 multicenter study of the GPOH].

After a pilot phase in 1988 the liver tumour study HB-89 of the German Society of Pediatric Oncology and Hematology (GPOH) was started on January 1st 1989. It comprises the malignant epithelial liver tumours of childhood--hepatoblastoma and hepatocellular carcinoma. The objectives of the study are 1. to reduce surgical complications and incomplete tumour resections by restricting the primary operation to a lobectomy of the liver in case of small tumours or to a biopsy in case of extended disease, and 2. to reduce the size of large tumours by means of chemotherapy for subsequent resection. The tumourreductive and adjuvant chemotherapy consists of the combinations of ifosfamide, cisplatinum, adriamycin (IPA) and cisplatinum plus adriamycin as continuous infusion (PA-cont). Up to December 31st 1991 a total of 78 pediatric liver tumours were registered, 51 of those hepatoblastomas and 10 hepatocellular carcinomas. 19 (37%) hepatoblastomas were resected at primary operation (stage I and II). After chemotherapy 24 (83%) of 29 primarily inoperable hepatoblastomas (stage III and IV) became resectable, one child received a liver transplant. At the time all hepatoblastoma patients of stages I and II and 20 (69%) of stages III and IV are in remission, the overall remission rate is 81% (39/48). Seven patients with multifocal hepatoblastoma had an unfavourable outcome, only two are free of tumour. Of 10 patients with a hepatocellular carcinoma only three with a primary complete tumour resection are disease free. The IPA and PA-cont therapies were highly effective on hepatoblastomas (96%), but not on hepatocellular carcinomas.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMedDate not supplied

Pancreatic pseudo-cyst in children: echographic and angiographic demonstration.

Explore the source record for details and available documents.

Age Factors↗

PubMedDate not supplied

[Results of the HB-89 Study in treatment of malignant epithelial liver tumors in childhood and concept of a new HB-94 protocol].

94 children with a primary liver neoplasm were registered in the Cooperative Pediatric Liver Tumor Study HB-89 of the GPOH from 1988 to 1992. 64 of these had a hepatoblastoma (HB), 12 a hepatocellular carcinoma (HCC), 2 a sarcoma and 16 a benign tumor. 51 (80%) patients with an HB were 6 to 36 months of age, 9 with an HCC above 10 years. Initial serum-alpha-fetoprotein (AFP) was elevated in 51 HB patients and exceeded the 3-fold of normal range in 45. Children with low (< 100 ng/ml) and very high (> 1,000,000 ng/ml) levels had a significantly worse prognosis than those with intermediate values (p = 0.0014). AFP was moderately elevated in 9 HCC patients. All other tumor markers were only irregularly above normal range. 48 (77%) of HB patients survived, 45 (73%) are tumor free. All of 18 stage I and 4/5 stage II patients are in remission. After chemotherapy 30/36 stage III HBs and 2/5 stage IV HBs could be resected, 9 of these patients suffered from tumor relapse. Disease-free survival was 100% for stage I, 80% for stage II, 68% for stage III and zero for stage IV HB (p = 0.0005). Surgical complications occurred after 8% of biopsies, 13% of limited primary resections and 25% of extended secondary resections. There was no perioperative death. The completeness of tumor resection at primary or delayed surgery correlated significantly with patients disease-free survival (p < 0.0001). Chemotherapy with ifosfamide, cisplatin and adriamycin was effective in HB. 203 courses were given in 61 patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Biomarkers, Tumor↗