Primary sarcoma of the liver (PSL) is a rare entity accounting for less than 1% of all liver cancers, with unknown aetiology. Several subtypes have been recognized, with histology playing an essential role in patient tailoring and management. We are about to report a case of an unusual PSL, with peculiar morphologic and immunohistochemical properties. A 65-year-old female with a multicystic hepatic lesion underwent surgery due to spontaneous rupture. Pathology revealed dilated bile duct-like structures lined by benign cuboidal/columnar epithelium surrounded by neoplastic proliferation of spindle and epithelioid cells with interspersed osteoclast-like multinucleated giant cells, loosely arranged in a storiform pattern, positive for CD10. The conjugation of morphology and immunohistochemistry results provided the diagnosis of primary biliary adenosarcoma of the liver, biliary type. The patient experienced tumour relapse and died of disease 2 years and 7 months later.

肝原发性肉瘤（PSL）是一种罕见的实体，占所有肝癌的不到1％，病因未知。已经认识到几种亚型，组织学在患者剪裁和管理中起着至关重要的作用。我们将报告一例异常的PSL，具有特殊的形态学和免疫组化特性。一名65岁女性患有多囊性肝病，由于自发性破裂而接受了手术。病理显示胆管样结构扩张，周围排列着良性立方/柱状上皮，梭形和上皮样细胞瘤增生，周围散布着破骨细胞样多核巨细胞，散布成星形状，对CD10呈阳性。形态学和免疫组化结果的结合提供了肝原发性胆道腺肉瘤的诊断。患者经历了肿瘤复发并在2年零7个月后死于疾病。