Department of Pathology, Dr. Rela Institute & Medical Centre, Chennai, Tamil Nadu, India.
The Institute of Liver Disease & Transplantation, Dr. Rela Institute & Medical Centre, Chennai, Tamil Nadu, India.
Fetal Pediatr Pathol. 2023 Aug;42(4):709-718. doi: 10.1080/15513815.2023.2201318. Epub 2023 Apr 18.
Hepatocellular carcinoma (HCC) in pediatrics has a uniformly poor prognosis. Complete surgical resection or liver transplantation remain the only curative options. In contrast to adult HCC, literature on pediatric HCC is sparse and a majority of the distinct subtypes are undefined with regards to their histology, immunohistochemistry and prognosis.
Two infants, one with biliary atresia and another with transaldolase deficiency, underwent living donor liver transplants. Explant-liver histopathology revealed tumor with diffuse neoplastic syncytial giant cell pattern. Immunophenotypic characterization highlighted expression of epithelial cell adhesion molecule, alpha fetoprotein and metallothionein.
HCC with syncytial giant cells variant can occur in infants with underlying liver disease, specifically in our experience, with biliary atresia and another with transaldolase deficiency.
儿科肝细胞癌（HCC）的预后普遍较差。完全手术切除或肝移植仍然是唯一的治愈方法。与成人 HCC 相比，儿科 HCC 的文献很少，大多数不同的亚型在其组织学、免疫组织化学和预后方面尚未确定。
两名婴儿，一名患有胆道闭锁，另一名患有转醛醇酶缺乏症，接受了活体供肝移植。移植肝组织病理学显示弥漫性肿瘤性合胞巨细胞模式的肿瘤。免疫表型特征突出了上皮细胞黏附分子、甲胎蛋白和金属硫蛋白的表达。
合胞巨细胞变异型 HCC 可发生于患有基础肝病的婴儿中，特别是在我们的经验中，与胆道闭锁和另一种转醛醇酶缺乏症有关。
