具有多谱系分化的畸胎样肝母细胞瘤：一种罕见的组织学变异型及文献复习
Teratoid Hepatoblastoma with Multi-Lineage Differentiation: An Uncommon Histological Variant and Review of Literature.
作者信息
Parkhi Mayur, Mitra Suvradeep, Chatterjee Debajyoti, Peters Nitin J
机构信息
Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
Pediatric Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.
出版信息
J Clin Exp Hepatol. 2022 Sep-Oct;12(5):1393-1401. doi: 10.1016/j.jceh.2022.04.005. Epub 2022 Apr 10.
BACKGROUND
Teratoid hepatoblastoma is an uncommon but well-recognized variant of mixed hepatoblastoma.
CASE REPORT
A one-year female child presented with palpable and progressively increasing right abdominal mass for 3 months. The contrast-enhancing computed tomography (CECT) abdomen revealed a large heterogeneous hepatic mass measuring 12 × 6.6 × 6 cm. Histopathological examination of the resected specimen showed a mixed hepatoblastoma (epithelial and mesenchymal) with teratoid features and multi-lineage differentiation (all three germ cell layers). A focus showed embryonal rhabdomyosarcomatous element.
CONCLUSION
Teratoid hepatoblastoma can show a wide range of heterologous differentiation that may pose a significant diagnostic dilemma. uch a broad spectrum has not been described in the literature previously. An appropriate immunohistochemical panel may be needed to identify and delineate the various heterologous differentiation to clinch the correct diagnosis. Secondary somatic malignancy such as rhabdomyosarcoma can develop in a teratomatous element.
背景
畸胎样肝母细胞瘤是混合性肝母细胞瘤中一种罕见但已被充分认识的变异型。
病例报告
一名1岁女童出现可触及且逐渐增大的右腹部肿块3个月。腹部增强计算机断层扫描（CECT）显示一个大小为12×6.6×6 cm的巨大异质性肝脏肿块。切除标本的组织病理学检查显示为具有畸胎样特征和多谱系分化（所有三个胚层）的混合性肝母细胞瘤（上皮性和间叶性）。一个病灶显示胚胎性横纹肌肉瘤成分。
结论
畸胎样肝母细胞瘤可表现出广泛的异源性分化，这可能带来重大的诊断难题。此前文献中尚未描述过如此广泛的谱系。可能需要合适的免疫组织化学检测组合来识别和界定各种异源性分化以做出正确诊断。继发性躯体恶性肿瘤如横纹肌肉瘤可在畸胎瘤成分中发生。
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