Abstract
Hepatoblastoma is a rare pathology in pediatric patients, occurring in 5-10/one million children. A two year old patient was admitted to our clinic for intense abdominal pain. On examination he presented a large mass in the right hypochondrium. Abdominal ultrasound and computer tomography (CT) revealed a large, solid hepatic tumor with thrombosis of the right portal vein. Serum alpha fetoprotein (AFP) level and thrombocyte number were increased. Liver biopsy was performed and the histopathological aspect was specific for an epithelial hepatoblastoma. Chemotherapy was initiated, followed by surgery in two steps: right portal branch ligation and subsequent tumor resection. Six months after surgery a relapse was suspected, confirmed by CT and elevated AFP levels. A „rescue” liver transplantation was performed and the patient is disease-free for three years. Elevated AFP and thrombocyte levels point to the diagnosis of hepatoblastoma. Liver transplantation is the final option for extensive, inoperable or relapsing tumors.
Highlights
Hepatoblastoma is a rare malignancy, accounting for just over 1% of total pediatric cancers [1] and occurs in 5-10/one million children per year [2].The etiology of the disease is unknown
Whole-genome DNA analysis led to the detection of many altered genomic regions, of which gain of material on chromosome 2q 13-22, 2q 36-37 and deletions of 2p and 4q were associated with advanced tumors and poor prognosis [3,4]
The diagnosis of hepatoblastoma is suspected in the patient aged between 6 months and 3 years, in the presence of a hepatic tumor, thrombocytosis and a high level of serum alpha-fetoprotein (AFP) [3]
Summary
Hepatoblastoma is a rare malignancy, accounting for just over 1% of total pediatric cancers [1] and occurs in 5-10/one million children per year [2]. The diagnosis of hepatoblastoma is suspected in the patient aged between 6 months and 3 years, in the presence of a hepatic tumor, thrombocytosis and a high level of serum alpha-fetoprotein (AFP) [3]. Pure epithelial tumors account for approximately 56% of cases; they contain varying amounts of fetal cells, embryonal cells, or both. Within this group, purely fetal tumors account for 31% of hepatoblastomas and embryonal tumors account for 19% of hepatoblastomas [5]. The American study group recommends surgical intervention at the moment of diagnosis (applicable in 50% of patients) followed by post-surgical chemotherapy [7]. Informed written consent was obtained from the patient’s mother before the publication of this case report
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