Case Report: Hepatoblastoma with spindle cell sarcomatous metastasis in a 14-year-old girl

TL;DR

A 14-year-old girl with hepatoblastoma developed resistant metastasis, requiring a combination of chemotherapy and targeted therapy for disease stabilization.

Contribution

This case report highlights the aggressive and chemoresistant nature of mesenchymal hepatoblastoma metastasis and the potential of combined targeted therapy.

Findings

Mixed epithelial–mesenchymal hepatoblastoma was confirmed through surgical resection and pathology.

Metastatic spindle cell sarcoma showed resistance to multiple chemotherapy regimens.

Combination therapy with anlotinib and radiotherapy achieved disease stabilization.

Abstract

Hepatoblastoma (HB) is the most common pediatric liver malignancy and occurs predominantly in children younger than 5 years of age. We report the case of a 14-year-old girl who was diagnosed with mixed epithelial–mesenchymal hepatoblastoma without any metastases. Initial evaluation revealed a markedly elevated serum alpha-fetoprotein level (7,800 ng/mL) and a large hepatic mass (17.3 cm × 18.4 cm × 8.5 cm) in the left liver lobe on contrast-enhanced CT. The patient underwent surgical resection, and pathological examination confirmed mixed epithelial–mesenchymal HB. Postoperative chemotherapy (consisting of cisplatin, doxorubicin, and 5-FU) normalized serum alpha-fetoprotein levels; however, skull metastasis developed during treatment. Histopathological analysis of the metastatic lesion revealed spindle cell sarcoma with decreased GPC-3/Hepa expression and elevated CD34/Ki-67 expression. Multiple chemotherapy regimens (ifosfamide, carboplatin, and etoposide; and doxorubicin, vincristine, cyclophosphamide, and cisplatin) demonstrated limited efficacy. Subsequent treatment with alternating albumin-paclitaxel, gemcitabine, ifosfamide, and etoposide/cyclophosphamide, irinotecan, and vincristine chemotherapy combined with anlotinib and cranial radiotherapy achieved disease stabilization, with no subsequent progression observed during follow-up. This case highlights the aggressive nature and chemoresistance of the mesenchymal components of HB, emphasizing the need for novel therapeutic approaches that incorporate targeted agents.

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