Zhang Shu-Hong, Chen Guang-Yong, Wei Lin
Department of Pathology, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
Liver Transplantation Center, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
Int J Surg Pathol. 2023 Jun;31(4):485-490. doi: 10.1177/10668969231171127. Epub 2023 Apr 25.
Infantile hepatic hemangioma and hepatoblastoma are the most common benign and malignant tumors of the liver in the neonatal and early childhood periods, respectively. However, the simultaneous occurrence of these 2 tumors in the same liver lesion is very rare. We report a case of a newborn infant diagnosed with a liver mass by ultrasound examination 4 days after birth. Serum alpha-fetoprotein (AFP) was elevated for his age (32,881.7 ng/mL). The liver mass was resected. Macroscopically, an externally protruding mass measuring 6 × 4 × 3.5 cm was identified. Microscopically, we observed the coexistence of infantile hepatic hemangioma and epithelial hepatoblastoma components within the tumor. The infantile hepatic hemangioma component was composed of multiple small vascular channels lined by endothelial cells. In the hepatoblastoma component, tumor cells were arranged in a 2- to 3-cell-thick trabecular formation. Immunohistochemistry indicated that the tumor cells in the infantile hepatic hemangioma component expressed CD34, CD31, FLI1, and ERG, and those in the hepatoblastoma component expressed hepatocyte, keratin AE1/AE3 and keratin 8, glypican 3, glutamine synthetase, and AFP. Pathological examination confirmed the presence of an infantile hepatic hemangioma combined with epithelial hepatoblastoma (fetal type). The boy did not undergo chemotherapy after the operation. Regular follow-up through serum AFP levels and liver ultrasound for 16 months to date show that the serum AFP levels decreased continuously to normal levels, with no signs of tumor recurrence or metastasis. The coexistence of infantile hepatic hemangioma and hepatoblastoma is rare. Hepatoblastoma should be considered in neonates with liver tumors and elevated AFP.
婴儿肝血管瘤和肝母细胞瘤分别是新生儿期和幼儿期最常见的肝脏良性和恶性肿瘤。然而,这两种肿瘤同时出现在同一肝脏病变中非常罕见。我们报告一例新生儿病例,该患儿出生后4天经超声检查诊断为肝脏肿块。其血清甲胎蛋白(AFP)水平高于其年龄对应的正常范围(32,881.7 ng/mL)。肝脏肿块被切除。大体上,发现一个向外突出的肿块,大小为6×4×3.5 cm。显微镜下,我们观察到肿瘤内存在婴儿肝血管瘤和上皮性肝母细胞瘤成分。婴儿肝血管瘤成分由多个内衬内皮细胞的小血管腔组成。在肝母细胞瘤成分中,肿瘤细胞呈2至3层细胞厚的小梁状排列。免疫组织化学显示,婴儿肝血管瘤成分中的肿瘤细胞表达CD34、CD31、FLI1和ERG,肝母细胞瘤成分中的肿瘤细胞表达肝细胞、角蛋白AE1/AE3、角蛋白8、磷脂酰肌醇蛋白聚糖3、谷氨酰胺合成酶和AFP。病理检查证实存在婴儿肝血管瘤合并上皮性肝母细胞瘤(胎儿型)。该男孩术后未接受化疗。迄今为止,通过血清AFP水平和肝脏超声进行了16个月的定期随访,结果显示血清AFP水平持续下降至正常水平,无肿瘤复发或转移迹象。婴儿肝血管瘤和肝母细胞瘤同时存在的情况罕见。对于肝脏肿瘤且AFP升高的新生儿应考虑肝母细胞瘤。