Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India.
Department of Pathology and Laboratory Medicine, A.I.I.M.S., Bibinagar, Hyderabad, India.
Int J Surg Pathol. 2023 Dec;31(8):1618-1625. doi: 10.1177/10668969231157777. Epub 2023 Apr 3.
Yolk sac tumor is a malignant germ cell tumor, which typically occurs in the gonads with elevated serum alpha-fetoprotein (AFP). Among extragonadal sites, the liver is an uncommon location for primary pediatric yolk sac tumors. Other common hepatic tumors in this age group presenting with elevated serum AFP like hepatoblastoma and hepatocellular carcinoma must be differentiated from yolk sac tumors for initiating appropriate treatment and accurate prognostication. Lung metastasis with refractoriness to chemotherapy is an extraordinary presentation that has never been documented in the literature. We report our experience with a 2-year-old female child initially misdiagnosed as hepatoblastoma. It was found that LIN28 positivity by immunohistochemistry aided in confirmation of the histopathological diagnosis of primary yolk sac tumor of the liver.
卵黄囊瘤是一种恶性生殖细胞肿瘤,通常发生在血清甲胎蛋白(AFP)升高的性腺中。在性腺外部位,肝脏是儿童原发性卵黄囊瘤的罕见部位。其他在该年龄段中,血清 AFP 升高的常见肝肿瘤,如肝母细胞瘤和肝细胞癌,必须与卵黄囊瘤相鉴别,以便开始进行适当的治疗和准确的预后判断。化疗耐药性肺转移是一种罕见的表现,在文献中从未有过记载。我们报告了一例 2 岁女性患儿的经验,该患儿最初被误诊为肝母细胞瘤。免疫组织化学检测 LIN28 阳性有助于确认肝脏原发性卵黄囊瘤的组织病理学诊断。