Kwait Brittany, Paracha Awais, Durrani Umar, Attanasi Michael, Hussain Fahad, Wright Jervon, Lee Amanda, Papale Anthony, John Veena
Northwell Health, 300 Community Drive Suite 4DSU, Manhasset, NY, 11030, USA.
St. Louis University Medical Center, St. Louis, MO, USA.
J Gastrointest Cancer. 2025 Jun 11;56(1):131. doi: 10.1007/s12029-025-01250-8.
Primary hepatic yolk sac tumor (HYST) is a rare, extragonadal germ cell neoplasm that arises in hepatic tissue. This review aims to examine clinical features, diagnostic workup, and medical and surgical interventions in patients diagnosed with primary HYST.
A literature search was performed using PubMed from 1950 to June 2024 using the filters English language and full text along with keywords "Yolk sac tumor of the liver," "Endodermal Sinus tumor of liver," "Hepatic Yolk sac tumor," and "Hepatic Endodermal sinus tumor."
Our results examined 20 patients ranging from age 15 months to 64 years. The cases included 14 female and 6 male patients. The most common presenting symptom was abdominal pain in adults and abdominal distention in children. Upon histologic evaluation, Schiller-Duval bodies were seen in the majority of examined tumors. Commonly noted markers included alpha fetal protein, cytokeratin AE1/AE3, alpha-1 anti-trypsin, placental alkaline phosphatase, SALL4, and periodic acid-Schiff. Treatment options included chemotherapy, surgery, transplant, and trans-arterial chemoembolization.
In this review, we discuss clinical signs of primary HYST. We highlight the importance of Schiller-Duval bodies on histology, as well as new markers in an attempt to aid in making a diagnosis of HYST.
原发性肝卵黄囊瘤（HYST）是一种罕见的、发生于肝组织的性腺外生殖细胞肿瘤。本综述旨在探讨原发性HYST患者的临床特征、诊断检查以及药物和手术干预措施。
利用PubMed进行文献检索，检索时间范围为1950年至2024年6月，检索条件为英文文献、全文，并使用关键词“肝脏卵黄囊瘤”、“肝脏内胚窦瘤”、“肝卵黄囊瘤”和“肝内胚窦瘤”。
我们的研究结果纳入了20例患者，年龄从15个月至64岁不等。其中包括14例女性患者和6例男性患者。最常见的症状在成人中是腹痛，在儿童中是腹胀。经组织学评估，大多数检查的肿瘤中可见席勒-杜瓦尔小体。常见的标记物包括甲胎蛋白、细胞角蛋白AE1/AE3、α-1抗胰蛋白酶、胎盘碱性磷酸酶、SALL4和过碘酸希夫反应。治疗选择包括化疗、手术、移植和经动脉化疗栓塞。
在本综述中，我们讨论了原发性HYST的临床体征。我们强调了席勒-杜瓦尔小体在组织学上的重要性，以及有助于诊断HYST的新标记物。
