Primary Yolk Sac Tumor of the Liver in an Adult Man

Abstract

Primary yolk sac tumor of the liver is extremely rare in adults. We report a case of a young man with an unresectable primary yolk sac tumor of the liver, who had a platinum-refractory disease that progressed despite 2 lines of chemotherapy. We review the literature pertaining to primary yolk sac tumor of the liver and its management.

Liver transplantation for advanced-stage primary hepatic yolk sac tumor: A case report and literature review

“…It may be beneficial to preoperatively differentiate HCC, hepatoblastoma, and primary hepatic YST, as the latter 2 respond well to systemic chemotherapy. [6,[14][15][16]22] Surgical resection is the established curative treatment for primary hepatic YST. The successful removal of tumors combined with preoperative or postoperative platinum-based chemotherapy [6,10,12,14,15] has been implemented in several patients with primary hepatic YST.…”

“…Most patients die within 1 year due to widespread disease [3,4,10] or a lack of follow-up outcomes. [5,9,[14][15][16][17] Limited data suggest that liver transplantation is an alternative treatment for primary hepatic YST. [11,23] In this study, we report a case of multiple unresectable hepatic YSTs with extrahepatic metastasis that underwent successful liver transplantation with concurrent surgical removal of the metastasis.…”

“…only 15 adult cases reported to date in the English literature (Table 1). [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17] Owing to the lack of specific laboratory biomarkers and imaging manifestations, it is difficult to differentiate primary hepatic YST from hepatocellular carcinoma (HCC) and hepatoblastoma. Most primary hepatic YSTs are diagnosed by incidental preoperative biopsy or postoperative histopathological examination, and are characterized by the formation of the Shiller-Duval body, that is, central vessels surrounded by epithelial tumor cells and fibrous tissue.…”

Primary hepatic mixed germ cell tumor in an adult

“…The incidence is bimodal, and peaks at around 2 years of age, with another peak after puberty [ 2 ]. Extragonadal GCTs commonly arise from midline structures, including the mediastinum and retroperitoneum [ 6 ].…”

“…To the best of our knowledge, this is the first report of mixed GCT composed of choriocarcinoma and yolk sac tumors that originated in the liver of an adult. To date, only 13 cases of primary hepatic GCTs in adult case reports or case series have been reported in the literature, all of which are summarized in Table 1 [4,6,8,[11][12][13][14][15][16][17][18][19]. Primary hepatic GCTs predominantly affect males with a male to female ratio of 2:1.…”