Discussion
Several clinical studies reporting hepatoblastomas have constructed a
therapeutic strategy consiting mainly of chemothrapy and surgery6)-9). A chemotherapy course composed of platinum and
anthracycline has been used as a standard treatment 6),
7). An appropriate chemotherapeutic regimen for paeditric patients with
HCC has not been established because the number of the cases is low and
pathological findings are different from that of adults. Indeed,
progonosis for the patients remains poor 10). In
parallel, HCCs in paediatric patients may not be biologically similar to
HCCs in adult patients 1). In the current case,
pathological examination review revealed a malignant hepatic tumour
consisting of HCC and hepatoblastoma. That is, the tumour had regions of
HCC and lesions of hepatoblastoma. Prokurat et al. 4)and Ozcan et al. 5) reported cases of the hepatic
tumours, such as transitional liver cell tumours, which were structured
by the components of both HCC and hepatoblastoma.
In the current case, CITA chemotherapy decreased the AFP level slowly,
and lesions by CT resolved. It suggested that CITA was effective for the
components of hepatoblastoma. Then, the patient’s serum AFP increased
again and it was caused by enlargement of HCC component, which was
revealed by the pathological findings of the specimen obtained at the
second surgery. Lenvatinib has recently been approved for treatment of
HCCs in Japan because it was reported that lenvatinib was non-inferior
to sorafenib in overall survival in advanced HCC in adult patients3). Conceivably, administering a multikinase
inhibitor, such as lenvatinib, combimed with CITA, might be able to
control HCC components although the utility of this combination was not
reported previously. We tried lenvatinib after relapse of the disease
and a positive effect was not observed.
Genomic variations have also been revealed in hepatic tumours11), 12). In the current case, the results of the
oncogene panel did not show any actionable variation. ATRX splice site
mutation observed in our patient was reported in also low-grade glioma13), but not in HCC. Differential analysis of a panel
of oncogenic mutations may clarify the similarity and difference in
hepatic tumors of children and adults.