[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)
von Schweinitz
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Kinderchirurgische Abteilung, Medizinischen Hochschule Hannover.
Bürger
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D
Bode
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Weinel
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P
Erttmann
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R
Hecker
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H
Mildenberger
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H
D014408:Biomarkers, Tumor / Q000097:blood; D006528:Carcinoma, Hepatocellular / Q000188:drug therapy / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006498:Hepatectomy; D018197:Hepatoblastoma / Q000188:drug therapy / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D008297:Male; D009367:Neoplasm Staging; D012509:Sarcoma / Q000188:drug therapy / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D015996:Survival Rate; D000509:alpha-Fetoproteins / Q000378:metabolism
D014408:Biomarkers, Tumor; D000509:alpha-Fetoproteins