Adult hepatoblastoma: Learning from children

Abstract

Hepatoblastoma is the most common malignant liver tumour in infants and young children. Its occurrence in the adult population is debated and has been questioned. The aim of this paper is to review the histological and clinical features of adult hepatoblastoma as described in the adult literature, and to compare the findings with those of paediatric hepatoblastoma. The developmental and molecular aspects of hepatoblastoma are reviewed and their potential contribution to diagnosis of adult hepatoblastoma discussed. Case reports of adult hepatoblastoma identified by a PubMed search of the English, French, German, Italian, and Spanish literature through March 2011 were reviewed. Forty-five cases of hepatoblastoma were collected. Age at presentation was variable. Survival was uniformly poor, except for the rare patients who presented with the relatively differentiated, foetal type. The common denominator between adult and paediatric cases is the occurrence of embryonal or immature aspect of the tumours. Whether the adult cases of hepatoblastoma represent blastemal tumours, stem cell tumours, or unusual differentiation patterns in otherwise more frequent adult liver tumours remains to be established. Adult tumours labelled as hepatoblastoma are characterised by malignant appearing mesenchymal components. Surgical management is the cornerstone of therapy in children and also appears to confer an improved prognosis in adults. Whether adult hepatoblastoma exists, remains controversial. Indeed, several features described in adult cases are markedly different from hepatoblastoma as it is understood in children, and other differential diagnoses should also be entertained. Nonetheless, hepatoblastoma should be considered in adults presenting with primary liver tumours in the absence of pre-existing liver disease. Adult and paediatric patients with immature hepatoblastoma appear to have worse outcomes, and adults presenting with presumed hepatoblastoma have an overall poorer prognosis than children with hepatoblastoma. In all patients, surgery should be the treatment of choice, neoadjuvant chemotherapy is advisable.

Adult hepatoblastoma

“…The first case of an adult hepatoblastoma was described in 1958 (8,9), having been reported 45 cases up to June 2012 and recently collected by Rougemont (1). Similarly as the pediatric cases, they usually present as a right upper quadrant mass, generally asymptomatic, and without previous history of liver disease, although hepatitis virus infection (HAV, HBV, HCV) has been reported in 25 % of the adult cases, as occurred in our case (1,2).…”

“…Several hypothesis have been raised regarding its embryo origin and the role of pluripotent hepatic stem cells as well (3,4). We report herein the third case of HBA in our country, and the 46 th in the literature (1,5,6).…”

“…Depending on the mesenchymal composition, areas of hemorrhage and necrosis can be revealed. In the mixed hepatoblastomas (epithelial and mesenchymal) -45 % of AHB-, osteoid and cartilage tissue can be identified showing a teratogenic pattern (1,10). The AHB histology has been compared with the pediatric hepatoblastoma.…”

“…Different mesenchymal components resembling fibrosarcoma, osteosarcoma, chondrosarcoma, angiosarcoma and rhabdomyosarcoma features have been described (1,10).…”

“…Forty five cases have been described up to may 2012 (1). In contrast to the children hepatoblastoma (HBI) it has a fatal prognosis, with a mean survival time of two months and one-year survival of 24 % (1,2).…”