Spectrum of appearances on CT and MRI of hepatic epithelioid hemangioendothelioma

Zhou, Lisha; Cui, Min; Xiong, Juxin J.; Dong, Zhi; Luo, Yanji; Xiao, Huangmeng; Xu, Ling; Huang, Kun; Li, Zi Ping Z.P.; Feng, Shi Ting

doi:10.1186/s12876-015-0299-x

Cited by 50 publications

(70 citation statements)

References 17 publications

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“…Hepatic EHE routinely presents as multiple nodules involving both lobes of the liver with a predominantly peripheral or subcapsular growth pattern. 4,6,7,12,13 Late in the disease process, the individual lesions may grow and coalesce into larger, more-complex masses. Hepatic EHE is sometimes stratified into nodular and diffuse types, based on those early and late appearances, respectively.…”

Section: Imaging and Gross Pathologymentioning

confidence: 99%

“…The literature reports that 27% to 37% of patients have extrahepatic spread at their time of diagnosis. 4,12,14 On noncontrast computed tomography imaging, the tumor nodules most commonly have a hypodense appearance, and subcapsular lesions may show associated capsular retraction. Calcifications, compensatory hypertrophy, or cysts are less commonly seen.…”

Section: Imaging and Gross Pathologymentioning

confidence: 99%

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Hepatic Epithelioid Hemangioendothelioma

Studer¹,

Selby²

2018

Archives of Pathology &Amp; Laboratory Medicine

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Epithelioid hemangioendothelioma is a rare vascular tumor, composed of epithelioid and histiocytoid vascular endothelial cells in myxoid or fibrotic stroma, which can arise in multiple locations throughout the body. In the liver, this neoplasm usually presents on imaging as an incidental finding of multifocal, heterogeneously enhancing nodules in both lobes or presents clinically with nonspecific abdominal symptoms. Histologically, the tumor has been mistaken for metastatic carcinoma, angiosarcoma, hepatocellular carcinoma, and cholangiocarcinoma. The neoplasm usually stains positive for vascular markers, such as factor VIII-related antigen, CD31, and CD34, and negative for cytokeratins. The translocation t(1;3)(p36.3;q25), resulting in the CAMTA1- WWTR1 fusion product, is the most commonly identified genetic abnormality with this tumor. Although hepatic epithelioid hemangioendothelioma can have a varied clinical course, it is generally considered less aggressive than angiosarcoma. There is no consensus treatment protocol and techniques including liver transplantation, liver resection, chemotherapy and/or radiation therapy, and surveillance have all been used with varying outcomes.

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Section: Imaging and Gross Pathologymentioning

confidence: 99%

Section: Imaging and Gross Pathologymentioning

confidence: 99%

Hepatic Epithelioid Hemangioendothelioma

Studer¹,

Selby²

2018

Archives of Pathology &Amp; Laboratory Medicine

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“…Capsular retraction is a common finding. [64] In the literature, multifocal involvement (multifocal nodular and diffuse coalescent) (85%) is more common than unifocal involvement [62] (Fig. 10).…”

Section: Discussion and Reviewmentioning

confidence: 99%

“…[60] HEHE is hypoattenuated and calcification foci can be seen in about 20% patients on precontrast CT. [65] HEHE typically demonstrates hypointensity on T1-weighted images and heterogeneous hyperintensity on T2-weighted images, compared with the normal liver parenchyma, on MRI. [64] There are diversities of enhancing patterns on both postcontrast CT and MR studies, which include peripheral or target enhancement, with central hypoenhancement. HEHE occasionally may show a peripheral hypodense or hypointense rim, which correlates with the avascular rim seen under pathologic examination.…”

Section: Discussion and Reviewmentioning

confidence: 99%

Uncommon liver tumors

Chiu²,

Yeh³

et al. 2016

Medicine

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Background:Beside hepatocellular carcinoma, metastasis, and cholangiocarcinoma, the imaging findings of other relatively uncommon hepatic lesions are less discussed in the literature. Imaging diagnosis of these lesions is a daily challenge. In this article, we review the imaging characteristics of these neoplasms.Methods:From January 2003 to December 2014, 4746 patients underwent liver biopsy or hepatic surgical resection in our hospital. We reviewed the pathological database retrospectively. Imaging of these lesions was reviewed.Results:Imaging findings of uncommon hepatic lesions vary. We discuss the typical imaging characteristics with literature review. Clinical and pathological correlations are also described. Primary hepatic lymphoma consists only of 1% of the extranodal non-Hodgkin lymphoma, and is defined as the one involving only the liver and perihepatic lymph nodes within 6 months after diagnosis. Combined hepatocellular and cholangiocarcinoma (cHCC-CC) shares some overlapping imaging characteristics with both HCC and cholangiocarcinoma because of being an admixture of them. Angiosarcoma is the most common hepatic mesenchymal tumor and is hypervascular in nature. Inflammatory pseudotumor is often heterogeneous on ultrasonography and with enhanced septations and rims in the portovenous phase after contrast medium. Angiomyolipoma (AML) typically presents with macroscopic fat components with low signal on fat-saturated magnetic resonance imaging (MRI) and presence of drainage vessels. Intraductal papillary neoplasm of the bile duct (IPNB) is thought of as a counterpart to the pancreatic intraductal papillary mucinous neoplasm. Most of the IPNBs secrete mucin and cause disproportional dilatation of the bile ducts. Mucinous cystic neoplasm (MCN) contains proteinaceous and colloidal components without ductal communication and characterizes with hyperintensity on T1-weighted imaging. Other extremely rare lesions, including epithelioid hemangioendothelioma and inflammatory pseudotumor-like follicular dendritic cell sarcoma, are also discussed. Hepatoblastoma and mesenchymal hamartoma, mostly in children, are also briefly reviewed as well.Conclusion:It is important for radiologists to be familiar with the typical imaging features of the uncommon hepatic neoplasms. If imaging findings are not typical or diagnostic, further biopsy is required.

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“…For example, the lesions appear as two-layered "target-like" or "halo" sign on T2WI or DWI with gradual ring-like peripheral enhancement on contrast-enhanced CT or MRI. One of the typical features is "lollipop sign", formed by hepatic or portal vein and their branches heading towards and terminating the edge of nodules [3]. Blachar et al considered capsular retraction as an important and non-specific sign of HEHE, but mostly in nodules larger than 2.0 cm [4].…”

mentioning

confidence: 99%