原发性肝平滑肌肉瘤是一种罕见的肝脏原发性间充质肿瘤,需要排除任何其他原发灶,并在组织学和免疫组织化学上排除其他具有梭形细胞形态的肝/肝外肿瘤。英文文献中仅报道了约70例,其中许多肿瘤具有免疫抑制或相关恶性肿瘤形式的易感病症。这种肿瘤在免疫活性个体中的发生也是已知的。该肿瘤的组织形态学显示梭形细胞病变,需要与该区域的其他梭形细胞病变区分开。这种肿瘤的主要诊断挑战在于它的稀有性,在给定的地点缺乏意识和形态模拟者。需要完整范围的免疫组织化学标记以将病变与其接近的形态模拟物区分开。这里,我们讨论了一名成年女性患者的原发性肝平滑肌肉瘤病例,该病例具有详细的组织形态学差异和各自的免疫谱。
Primary hepatic leiomyosarcoma is a rare primary mesenchymal tumor of the liver requiring exclusion of any other primary site of origin and histological and immunohistochemical exclusion of other hepatic/extrahepatic tumors with spindle cell morphology. Only about 70 cases are reported in the English literature and many of these tumors have predisposing conditions in the form of immunosuppression or associated malignancies. The occurrence of this tumor in the immunocompetent individual is also known. Histomorphology of this tumor shows a spindle cell lesion which needs to be distinguished from other spindle cell lesions of this region. The main diagnostic challenge of this tumor lies in its rarity, lack of awareness and morphological mimickers in the given site. A complete range of immunohistochemical markers is required to distinguish the lesion from its close morphological mimickers. Here, we discuss a
case of primary hepatic leiomyosarcoma in an adult female patient with detailed histomorphological differentials and respective immunoprofiles.