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肝静脉流出道梗阻中的局灶性增生性肝细胞结节:4例患者及24个结节的临床病理研究

Focal hyperplastic hepatocellular nodules in hepatic venous outflow obstruction: a clinicopathological study of four patients and 24 nodules.

作者信息

Ibarrola C, Castellano V M, Colina F

机构信息

Pathology Department, University Hospital Doce de Octubre, Madrid, Spain.

出版信息

Histopathology. 2004 Feb;44(2):172-9. doi: 10.1111/j.1365-2559.2004.01795.x.

Abstract

AIMS

In hepatic venous outflow obstruction (Budd-Chiari syndrome), focal hepatocellular nodules are occasionally discovered showing variable morphology. These could be interpreted either as neoplastic (adenoma), regenerative (large regenerative nodule) or reactive to abnormal vasculature (focal nodular hyperplasia). The aim of this study was to investigate their histogenesis and to determine their morphological characteristics in order to provide diagnostic criteria.

MATERIAL AND METHODS

Twenty-four hepatocellular nodules were studied, which were found in three explanted livers and in one additional autopsied liver from four patients with Budd-Chiari syndrome. As controls, we employed three explanted livers without nodules from patients who also suffered from Budd-Chiari syndrome. We attempted to classify the nodules morphologically as either adenoma-like, large regenerative nodule or focal nodular hyperplasia-like, using criteria from the literature.

RESULTS

Out of the four cases, we observed two nodules in each of two livers, five in the third one and up to 15 in the remaining one. The size of the nodules ranged from 4 to 25 mm. Eleven nodules could be categorized as large regenerative nodules (two of them with a central scar), seven as focal nodular hyperplasia-like and six as adenoma-like. Some large regenerative nodules showed proliferated arteries with muscular hyperplasia similar to that seen in focal nodular hyperplasia. In the individual livers we could find nodules of various categories. Patchy or diffuse monoacinar regeneration was seen in most cases (six out of seven cases) in the macroscopically non-nodular liver parenchyma. In addition, thrombotic obstruction of portal vein branches was present in all except one of the nodular cases, but in none of the controls. Thus, it appears that portal venous obstructions are frequently, but not invariably associated with the development of nodules.

CONCLUSIONS

The hepatocellular nodules seen in livers from patients with Budd-Chiari syndrome share morphological characteristics with large regenerative nodules, focal nodular hyperplasia and hepatocellular adenomas. Their multiplicity, the existence of mixed lesions, the frequent hepatocellular regenerative background as well as the frequently associated portal venous obstructions suggest that these nodules are regenerative in nature and conditioned by an uneven blood perfusion throughout the liver. In their differential diagnosis, the clinicopathological context in which they occur is of paramount importance and should allow recognition that those resembling adenomas may not be true neoplasms.

摘要

目的

在肝静脉流出道梗阻(布加综合征)中,偶尔会发现形态各异的局灶性肝细胞结节。这些结节可被解释为肿瘤性(腺瘤)、再生性(大再生结节)或对异常血管的反应性(局灶性结节性增生)。本研究的目的是探讨其组织发生,并确定其形态特征以提供诊断标准。

材料与方法

研究了24个肝细胞结节,这些结节来自4例布加综合征患者的3个移植肝脏和1个尸检肝脏。作为对照,我们使用了3个同样患有布加综合征但无结节的移植肝脏。我们尝试根据文献标准将结节形态学分类为腺瘤样、大再生结节或局灶性结节性增生样。

结果

在这4例病例中,我们在其中2个肝脏中各观察到2个结节,在第3个肝脏中观察到5个结节,在其余1个肝脏中观察到多达15个结节。结节大小范围为4至25毫米。11个结节可归类为大再生结节(其中2个有中央瘢痕),7个为局灶性结节性增生样,6个为腺瘤样。一些大再生结节显示动脉增生伴肌肉增生,类似于局灶性结节性增生所见。在各个肝脏中,我们可以发现不同类别的结节。在宏观上无结节的肝实质中,大多数病例(7例中的6例)可见片状或弥漫性单腺泡再生。此外,除1例结节病例外,所有病例均存在门静脉分支的血栓性阻塞,但对照组均无此情况。因此,似乎门静脉阻塞经常但并非总是与结节的发生相关。

结论

布加综合征患者肝脏中所见的肝细胞结节具有与大再生结节、局灶性结节性增生和肝细胞腺瘤相同的形态特征。它们的多发性、混合性病变的存在、频繁的肝细胞再生背景以及经常伴发的门静脉阻塞表明这些结节本质上是再生性的,并且受整个肝脏血流灌注不均的影响。在它们的鉴别诊断中,它们发生的临床病理背景至关重要,并且应该能够认识到那些类似腺瘤的可能并非真正的肿瘤。

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