# 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.

## Key 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.