肝脏原发粒层细胞瘤伴海绵状血管瘤1例报道并文献复习

摘要

目的 探讨肝脏原发粒层细胞瘤(granulosa cell tumor)伴海绵状血管瘤的临床病理学特点.方法 对1例肝脏原发粒层细胞瘤伴海绵状血管瘤进行组织学观察和免疫组化染色,观察其超微结构及临床影像学特点,并复习相关文献.结果 患者女性,CT及MRI提示肝脏右叶可见一巨大实性肿物,低密度.肉眼见肿物突向肝脏下方,切面呈实性;另外,在肝脏膈面被膜下见一灰红色出血区,呈疏松海绵状.镜下巨大肿物中肿瘤细胞椭圆形或梭形,呈微滤泡状、小梁状排列,部分区域呈实性弥漫状分布,类似于卵巢粒层细胞;部分区域呈束状排列,间质中可见较多胶原纤维瘢痕形成,似孤立性纤维性肿瘤.肿瘤细胞核内可见小核仁,并可见纵形核沟.免疫组化结果示肿瘤细胞CD99、α-inhibin、CK8、CK18、SMA和BCL-2均(+),Ki-67增殖指数＜1%.超微结构显示肿瘤细胞可见各种形式的核皱叠形成,部分细胞呈Call-Exner小体样结构排列,偶见桥粒结构,提示该肿瘤与卵巢粒层细胞瘤极其相似.结论 粒层细胞瘤原发于肝脏且合并海绵状血管瘤,非常罕见,目前其临床生物学行为难以确定,需对患者进行长期随访.%Purpose To study the clinicapathological features of primary granulosa cell tumor of liver associated with cavernous hemangioma. Methods One case of primary granulosa cell tumor of liver associated with cavernous hemangioma was studied by histology, immunohistochemistry, ultrastructural analyses and clinical imaging. Results A female patient complained of a mass by CT and MRI. The tumor protruded into the lower portion of the liver, and the cut surface was solid. Diaphragmatic surface of liver shows an area of gray red bleeding and loose sponge-like. The tumor cells were oval or spindle-shaped arranged in micro-follicular or trabecular, some areas showed solid and diffuse distribution, which was similar to ovarian granulosa cells; some areas were arranged in bundles with interstitial collagen fibers, like solitary fibrous tumor. Small nucleoli and longitudinal or transverse nuclear grooves were seen in nuclei of the tumor cells. Immunohistochemically, they were positive for CD99, ct-inhibin, CK8 , CK18 , SMA and BCL-2, with Ki-67 prolifera-tive index less than 1%. Ultrastructural study showed: the formation of various forms of nuclear grooves were seen in the tumor cells, and some cells were arranged in Call-Exner bodies-like structures, occasionally desmosome was noted between the cells, suggesting that the tumor was very similar to ovarian granulosa cell tumor. Conclusions The primary granulosa cell tumor of liver associated with cavernous hemangioma is rare. The behavior of tumor is unpredictable and long-term follow up is nelessary.