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目的 :探讨肾脏不典型血管平滑肌脂肪瘤 (atypicalangiomyolipoma,AAML)的临床病理特征。 方法 :收集 4例曾误诊为肾肉瘤样癌或恶纤组的AAML行光镜、免疫组化观察 ,并对其中 1例和另 3例典型AML作电镜观察。结果 :AAML具有以下的病理特点 :①肉眼上切面呈棕褐色 ,可伴有坏死及出血 ;②光镜下表现为富细胞 ,主要由胞浆丰富嗜酸性的单核或多核多形性瘤巨细胞和梭形的肉瘤样细胞及胞浆透明成片状排列的上皮样细胞组成。缺乏成熟梭形平滑肌细胞 ,厚壁血管及脂肪细胞少或无 ;③巨核畸形核及核内胞浆包涵体较多见 ,但未见核分裂象 ;④免疫组化 :瘤细胞HMB45 (+)、CD6 8(+)、CK(- ) ,而SMA瘤巨细胞阴性 ;⑤电镜示瘤细胞胞浆内线粒体和糖原较丰富 ,并见直径约 10 0nm的密电子颗粒 ,未见明确的黑色素颗粒前体及成熟的黑色素颗粒。随访 6～ 36个月均健在。结论 :肾脏AAML可能仅在组织形态上呈肉瘤样特征 ,而实际仍属良性病变。其特征性的免疫组化表达及超微结构为其鉴别诊断提供了依据。
Objective: To investigate the clinicopathological features of atypicalangiomyolipoma (AAML). Methods: Four cases of AML with misdiagnosed as sarcomatoid carcinoma or malignant fibroids were collected and observed by immunohistochemistry. One and three cases of typical AML were observed by electron microscopy. Results: The pathological features of AAML were as follows: ① The upper cut of the human eye was tan, which could be associated with necrosis and hemorrhage. ② The cells under light microscopy showed rich cells mainly composed of abundant eosinophilic mononuclear or polymorphic tumor Cells and spindle-shaped sarcomatoid cells and cytoplasm transparent into a sheet-like epithelial-like cells. Lack of mature spindle smooth muscle cells, thick blood vessels and fat cells less or no; (3) megakaryocyte nucleus and nuclear cytoplasmic inclusion bodies more common, but no mitosis; Immunohistochemistry: tumor cells HMB45 (+), CD6 8 (+), CK (-), and SMA tumor giant cell negative; ⑤ electron microscopy tumor cells cytoplasm mitochondria and glycogen more abundant, and see the diameter of about 100nm dense electronic particles, no clear melanin particles Precursor and mature melanin granules. All patients were followed up for 6 ~ 36 months. CONCLUSIONS: Renal AAML may only exhibit sarcomatoid features in the morphology of the tumor, but in fact it is still a benign lesion. Its characteristic immunohistochemical expression and ultrastructure provide the basis for its differential diagnosis.