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肝脏血管平滑肌脂肪瘤:从 47 例 55 个肿瘤的临床病理发现重新评估形态特征,并描绘出新的特征性组织学特征。

Angiomyolipoma of the liver: a reappraisal of morphological features and delineation of new characteristic histological features from the clinicopathological findings of 55 tumours in 47 patients.

机构信息

Department of Diagnostic Pathology, Nara Medical University School of Medicine, Kasihara, Nara, Japan.

出版信息

Histopathology. 2012 Nov;61(5):863-80. doi: 10.1111/j.1365-2559.2012.04306.x. Epub 2012 Aug 8.

Abstract

AIMS

The aim of this study was to characterize and delineate the broad histological spectrum of hepatic angiomyolipoma (AML) and to obtain a better understanding of its clinicopathological diagnosis by reviewing a large series of AMLs.

METHODS AND RESULTS

According to the proportions of three histological components, AML could be classified into 10 types; 36 of 55 tumours (65%) were classified as myomatous, eight as myoangiomatous, six as mixed (conventional), two as lipomatous, two as myolipomatous, and one as lipomyomatous. The morphology of smooth muscle cells (SMCs) in AML was quite variable, giving a wide variety of growth patterns. Fourteen (25%) of 55 tumours showed severe cellular atypia, and invasive growth of tumour cells was found in 69% of the tumours. However, except for two autopsy cases, all of the patients were in good health at follow-up, without metastases. Immunostaining for HMB-45 was positive in all cases. We found frequent lymphocyte infiltration, and the occasional presence of abnormally large blood vessels around the tumour at the tumour-background liver interface, features which have not been reported previously.

CONCLUSIONS

The majority of hepatic AMLs were myomatous in type, showing variable cellular morphology and growth patterns. Cellular atypia and invasive growth were frequent, indicating that hepatic AMLs often show malignancy-like histological features. Although the majority of cases behave as benign tumours, AML should be considered to have uncertain malignant potential, and careful follow-up of patients is recommended. Immunostaining for HMB-45 is specific for AML, and establishes the diagnosis. The occasional presence of abnormally large blood vessels around the tumour may give new insights into the evaluation of findings from imaging.

摘要

目的

本研究旨在对肝脏血管平滑肌脂肪瘤(AML)的广泛组织学谱进行描述和分类,并通过回顾大量 AML 病例,更好地理解其临床病理诊断。

方法和结果

根据三种组织学成分的比例,AML 可分为 10 型;55 个肿瘤中有 36 个(65%)被归类为肌瘤型,8 个为肌血管瘤型,6 个为混合(常规)型,2 个为脂肪瘤型,2 个为肌脂瘤型,1 个为脂肪肌瘤型。AML 中平滑肌细胞(SMCs)的形态变化多样,表现出多种生长方式。14 个(25%)肿瘤显示严重的细胞异型性,69%的肿瘤中发现肿瘤细胞的侵袭性生长。然而,除了两例尸检病例外,所有患者在随访中均身体健康,无转移。所有病例的 HMB-45 免疫染色均为阳性。我们发现经常有淋巴细胞浸润,偶尔在肿瘤与背景肝交界处周围有异常大的血管,这些特征以前没有报道过。

结论

大多数肝 AML 为肌瘤型,具有不同的细胞形态和生长方式。细胞异型性和侵袭性生长很常见,表明肝 AML 通常具有类似于恶性的组织学特征。尽管大多数病例表现为良性肿瘤,但 AML 应被认为具有不确定的恶性潜能,建议对患者进行仔细随访。HMB-45 免疫染色对 AML 具有特异性,可明确诊断。偶尔在肿瘤周围存在异常大的血管可能为评估影像学发现提供新的见解。

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