Primary hepatic carcinoma with inhibin positivity is a rare aggressive liver tumor with seven cases described. The tumor presents at a younger age than primary hepatic carcinoma with all cases being females. RNA albumin ISH positivity suggests the tumor to be a primary hepatic carcinoma. The tumor is different from hepatocellular carcinoma as well as intrahepatic cholangiocarcinoma because of its distinct morphology, lack of hepatocellular differentiation, strong inhibin staining, and lack of typical mutations. A 26-year-old male presented with a 20-cm liver mass. The tumor progressed on therapy with development of multiple lung metastasis. Currently, the patient is enrolled in phase II clinical trial utilizing nivolumab and ipilumumab. While the tumor has a female preponderance, it is not exclusively found in females. Additional studies are necessary to determine the cause of inhibin staining, driving molecular alterations, natural history of this rare tumor, and to come up with consensus nomenclature.

具有抑制素阳性的原发性肝癌是一种罕见的侵袭性肝肿瘤，描述了7例病例。该肿瘤比原发性肝癌年轻，所有病例均为女性。RNA白蛋白ISH阳性表明该肿瘤为原发性肝癌。该肿瘤与肝细胞癌以及肝内胆管癌不同，因为其形态独特，肝细胞分化不足，抑制素染色强，缺乏典型突变。一名26岁的男性有20厘米的肝脏肿块。随着多发肺转移的发展，肿瘤在治疗上进展。目前，该患者正在接受使用nivolumab和ipilumumab的II期临床试验。尽管该肿瘤具有女性优势，但并非仅在女性中发现。