Institute of Pathology, University of Leipzig, Liebigstrasse 26, Leipzig, Germany.
Diagn Pathol. 2009 Nov 12;4:37. doi: 10.1186/1746-1596-4-37.
Primary liver tumors in children are rare with malignant hepatoblastoma being the most common neoplasm. In this report, we describe the diagnosis and clinical management of a large liver tumor in a 3-year-old child that displayed the features of both, conventional hepatoblastoma and malignant teratoma. Pathological assessment on a pre-operative bioptical specimen showed an immature teratoid tumor with no area of hepatoblastic differentiation present. Histological and immunohistological examination of the resected tumor specimen additionally showed tumor areas of very different differentiation pattern intermixed with each other, namely areas of hepatoblastoma-typical and neuroblastoma-like morphology as well as areas of rhadomyosarcomatous differentiation.After chemotherapy the tumor size increased and an extended right hemihepatectomy was performed. Post-operatively, the general condition of the child improved and adjuvant chemotherapy was started two weeks later. 36 months after initial diagnosis the patient is healthy, in good general condition, and without any sign of residual tumor disease.Overall, we describe the diagnosis and clinical management of a large liver tumor in a 3-year-old child that displayed the features of both, conventional hepatoblastoma and malignant teratoma and was designated as mixed hepatoblastoma and teratoma. Though mesenchymal tumor portions can occur within hepatoblastomas, most commonly osteoid or chondroid, our case is different as it presents a large spectrum of mesenchymal and epithelial differentiation pattern in most of the lesion.
儿童原发性肝脏肿瘤较为罕见,其中恶性肝母细胞瘤最为常见。本报告描述了一名 3 岁儿童巨大肝脏肿瘤的诊断和临床处理方法,该肿瘤兼具常规肝母细胞瘤和恶性畸胎瘤的特征。术前活检标本的病理评估显示为无肝母细胞分化区的未成熟畸胎瘤。切除肿瘤标本的组织学和免疫组织化学检查进一步显示,肿瘤内存在非常不同分化模式的区域相互混合,即具有肝母细胞瘤样和神经母细胞瘤样形态的区域以及横纹肌肉瘤样分化的区域。化疗后肿瘤大小增大,随后进行了扩大的右半肝切除术。术后患儿一般情况改善,两周后开始辅助化疗。初始诊断后 36 个月,患儿健康状况良好,无残留肿瘤疾病迹象。总之,我们描述了一名 3 岁儿童巨大肝脏肿瘤的诊断和临床处理方法,该肿瘤兼具常规肝母细胞瘤和恶性畸胎瘤的特征,并被诊断为混合性肝母细胞瘤和畸胎瘤。虽然间叶性肿瘤部分可出现在肝母细胞瘤中,最常见的是骨样或软骨样,但我们的病例不同,因为它在大部分病变中呈现出广泛的间叶性和上皮性分化模式。