肾上皮样血管平滑肌脂肪瘤——附4例报告及文献复习

为了观察分析肾上皮样血管平滑肌脂肪瘤（angiomyolipoma，AML）的临床病理学特征，进一步认识、评价其病变性质。对4例肾上皮样AML的组织形态学特征和免疫组织化学进行观察分析，并结合文献对其临床病理特征和生物学行为进行探讨。4例病例组织形态学的主要特征：均呈明显的上皮样分化；浸润破坏性生长并伴有不同程度的不典型性；肿瘤组织广泛坏死；免疫学形态表现为肿瘤细胞呈HMB45阳性；临床均呈多器官发病，远隔脏器转移；1例合并结节性硬化症，术后10个月因本病死亡。分析结果提示，明显上皮样分化的AML生物学行为与经典型肾AML不同特征在于浸润性生长方式；细胞学异形性；多器官发病或远隔脏器的转移和致死性，证明恶性上皮样AML的存在。

Epithelioid angiomyolipoma of the kidney:a report of 4 cases and literature review

The objective of this study was to investigate the clinicopathologic features of epithelioid angiomyolipoma of kidney (KAML) and the histologic criteria of epithelioid KAML. Four cases of epithelioid KAML with HE and immunohistochemistry staining were included. Its histopathological and immunohistological characteristics, biologic behaviors and related literatures have been analyzed and reviewed. Of the four cases of epithelioid KAML, significant perivascular epithelioid cells were presented with invasive hyperplasia of atypical pleomorphism, hyperchromatic nuclei with frequent mitotic figures, giant neoplasic cells and extensive hemorrhagic necrosis immunohistochemcial staining showing a positive HMB45 and Ki-67 in most neoplasic cells. Multiple organ invasion, partly with remote metastases and necrosis of organs were found in the 4 cases including 1 case of tuberous sclerosis and 1 case of death. In conclusion, the most differences between the epithelioid AML and classic AML of kidney are the rare biological behavior of invasive proliferation, histological features of atypical hyperplasia, mitosis and immunohistochemcial presentation. The existence of malignant epithelioid AML is strongly suggested by the features of the involvement of multiple organs or remote metastases, and all above clincohistopathology and biological behavior of KAML.