Clinical Characteristics and Prognosis of Patients with Hepatoblastoma.
- Author:
Min Young KIM
;
Dae Yeon KIM
;
Hyo Seop AHN
;
Chong Jai KIM
;
In One KIM
;
Sung Eun JUNG
;
Seong Cheol LEE
;
Kwi Won PARK
;
Woo Ki KIM
- Publication Type:Original Article
- Keywords:
Hepatoblastoma;
Curative Resection;
Neoadjuvant Chemotherapy;
Prognosis;
Thrombocytopenia
- MeSH:
Biopsy;
Chemotherapy, Adjuvant;
Classification;
Diagnosis;
Drug Therapy;
Hepatoblastoma*;
Humans;
Prognosis*;
Survival Rate;
Thrombocytopenia;
Tumor Burden
- From:Journal of the Korean Association of Pediatric Surgeons
1997;3(2):133-142
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Hepatoblastoma is a rare pediatric malignancy which frequently presents at an advanced unresectable stage, but improved resectability and survival is being reported with the help of neoadjuvant chemotherapy. Twenty biopsy proven hepatoblastoma patients were diagnosed and managed during the period between January, 1987 and June, 1995. Median age at diagnosis was 13 months (2mo~7yr 10mo) with M : F = 13 : 7. The histologic profile included 13 epithelial (5 fetal, 4 mixed, 1 embryonal, 3 undetermined), and 5 mixed mesenchymal and epithelial types. Chemotherapy effectively reduced the tumor volume with statistical significance (p=0.008) and was able to convert 7 out of 9 initially unresectable cases (78%) to resectable ones. Fourteen operations were done, 12 radical and 2 palliative with or without adjuvant chemotherapy. The whole population was followed with a median duration of 33 months and the median survival in the whole group was 26 months. The curative resection group displayed a 5 year survival rate of 61.1% and none in the non-curative group survived for more than 13 months (p=0.0001). In univariate analysis for prognostic factors, large tumor size at diagnosis and absence of thrombocytopenia was associated with poor survival, but these differences were not statistically significant. Pure fetal histology was not associated with better prognosis and this may be due to a different histologic classification. In this new era of neoadjuvant chemotherapy, the optimal management strategy for hepatoblastoma is still debated with radical surgical resection at earliest possible time being the final goal. For now, individualized approach appears to be the choice.
