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目的探讨血管平滑肌脂肪瘤(AML)、上皮样血管平滑肌脂肪瘤(EAML)的临床病理学及良、恶性的诊断和鉴别诊断。方法对10例AML及其亚型EAML进行临床病理学及免疫组化观察,随访和复习相关文献。结果经典型AML4例,肌瘤型1例,EAML5例。其中4例发生在肾、肝和子宫。镜检:瘤细胞上皮样、疏松、不规则结节状或片状或围绕血管生长,核圆、稍大,轻度异型性,核分裂象<1个/50HPF;发生于阔韧带的1例EAML,瘤细胞上皮样,核大、深染、核异型,可见核内包涵体,核分裂象>1个/10HPF,胞质明显嗜酸,肿瘤性坏死和肿瘤组织侵及同侧卵巢及盆腔壁;随访近5年,疑似局部复发和肝转移。其他病例均未见复发转移。免疫组化:HMB45、melanA和SMA(+),desmin部分(+),S-100蛋白、CD34、CK、EMA和CgA(-)。结论 AML大部分为良性病变,但瘤组织出现坏死、瘤细胞核的活性增加、核的不典型性和肾外扩散时应高度考虑为恶变。对EAML病例即使是良性形态也必须密切随访,同时应与相似形态的肿瘤鉴别。
Objective To investigate the clinical and pathological features of angiomyolipoma (AML) and epithelioid angiomyolipoma (EAML) and the diagnosis of benign and malignant diseases. Methods 10 cases of AML and its subtype EAML were observed by clinicopathological and immunohistochemical methods, followed up and reviewed the relevant literature. Results The classic AML4 cases, myoma type in 1 case, EAML5 cases. Four of these occurred in the kidneys, liver and uterus. Microscopic examination: Epithelial tumor cells, loose, irregular nodular or flaky or surrounding blood vessels, nuclear round, slightly larger, mild atypia, mitosis <1 / 50HPF; occurred in the broad ligament in 1 case of EAML , Tumor cells epithelial, nuclear large, stained, nuclear atypia, visible nuclear inclusions, mitotic> 1 / 10HPF, cytoplasm obvious acidophilic, tumor necrosis and tumor invasion of ipsilateral ovarian and pelvic wall; Follow-up nearly 5 years, suspected local recurrence and liver metastases. No other cases of recurrence and metastasis. Immunohistochemistry: HMB45, melanA and SMA (+), desmin fraction (+), S-100 protein, CD34, CK, EMA and CgA (-). Conclusions Most of AML are benign lesions. However, neoplasms are necrotic and the activity of tumor nuclei is increased. Atypical nuclearity and extrarenal diffusion should be highly considered malignant. EAML cases, even if the benign morphology must also be followed up closely, and should be similar to the identification of tumors.