血管平滑肌脂肪瘤10例临床病理分析

Angiomyolipoma:a clinicopathologic analysis of 10 cases

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 clinicopathological features of angniomylipoma （AML） and epithelioid angniomylipoma （EAML） , and their diagnosis and differential diagnosis. Methods Ten cases of AML and its subtypes EAML were studied with light microscopy and immunohistoehemical staining, following-up and review of the literature. Results There were 4 cases of classical type, 1 leiomyomatous type （fusiform and epitheloid cells） , and 5 epitheloid type. There were 4 tumors arising from the kidney, liver and uterine. The tumor cells showed epitheloid, loosely arranged, irregular nodules or a sheet pattern, and surrounded the vessels, and the tumor cells had round nuclei, mild atypia and rare mitosis （1/50 HPF） ; 1 case was arised from broad ligament, in which the tumor cells appeared epithelioid, large nuclei, atypia, intranuclear inclusion, and more mitotic figures （1/10 HPF） , and the plasma was acidophilia, with necrosis. The tumor invaded ovaries and cavitas pelvis of the same side. After followed up for 5 years, this case was suspicious recurrence with liver metastasis. But there was no recurrence and metastasis in other cases. Immunohistochemical staining showed the tumor cells were positive for HMB-45, Melan-A and SMA, partially for desmin, but negative for S-100 protain, CD34, CK, EMA, and CgA. Conclusions AML usually is a benign tumor, but malignant transformation should be considered if appearence of necrosis, increased nuclear activity and atypia and spread out of the kidney. Any EAML case should be followed intimately up even if it seems benign morphologically, and it is also differentially diagnosed with tumors with similar morphology.