Hepatoblastoma. A clinical and pathologic study of 54 cases
The clinical and pathologic features of 54 children with hepatoblastoma are reviewed. Their average age at diagnosis was 17 months (range: 4 months-4 1/2 years) and there was a predilection for males in a ratio of nearly 2:1. Unusual manifestations included isosexual precocity (two cases), hemihyper...
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Veröffentlicht in: | The American journal of surgical pathology 1982-12, Vol.6 (8), p.693-705 |
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Sprache: | eng |
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Zusammenfassung: | The clinical and pathologic features of 54 children with hepatoblastoma are reviewed. Their average age at diagnosis was 17 months (range: 4 months-4 1/2 years) and there was a predilection for males in a ratio of nearly 2:1. Unusual manifestations included isosexual precocity (two cases), hemihypertrophy and Budd-Chiari syndrome (one case), and synchronous Wilms' tumor (one case). Thirty-two of 49 conventional hepatoblastomas were subclassified as epithelial type (19 predominantly embryonal, 13 predominantly fetal), and 12 had mixed epithelial-mesenchymal histology. Pathologic material in five cases was too limited for reliable subclassification. Hepatoblastomas classified as anaplastic type had more primitive histology with a predominance of small- to medium-sized cells in diffuse sheets. The overall mortality was 76% and the average duration of disease from diagnosis to death was 8 months (range: 1 month-27 months). Complete surgical resection remains the key treatment for achieving long-term survival and was attempted in 33 of the 54 children (61%). There were 13 long-term survivors followed for an average of 9 years. The most common type of hepatoblastoma in these children was epithelial with a predominantly fetal pattern. None of the children with anaplastic tumors survived. |
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ISSN: | 0147-5185 |
DOI: | 10.1097/00000478-198212000-00001 |