Hepatic epithelioid haemangioendothelioma (HEHE): a diagnostic dilemma between haemangioma and angiosarcoma

Rosmalen, Belle V. van; Verheij, Joanne; Phoa, Saffire S.K.S.; Gulik, Thomas M. van

doi:10.1136/bcr-2017-220687

Cited by 6 publications

(2 citation statements)

References 24 publications

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“…Because of its uncertainty, HEHE is commonly misdiagnosed as primary or secondary liver cancer. The tumor generally affects patients of all ages, and the highest incidence is between 35 and 45 years old (5)(6)(7), with a male-tofemale ratio of 2:3 (8,9). Etiologic factors are currently unknown, and although several risk factors have been proposed, none has been confirmed to increase the risk of developing HEHE, including the hepatitis virus and chronic liver diseases (2,10).…”

Section: Introductionmentioning

confidence: 99%

Novel and Specific MRI Features Indicate the Clinical Features of Patients With Rare Hepatic Tumor Epithelioid Hemangioendothelioma

Zhang

Zhong

et al. 2022

Front. Oncol.

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ObjectiveTo investigate the MRI features and clinical significance of hepatic epithelioid hemangioendothelioma (HEHE).MethodsClinical records and MRI findings were retrospectively evaluated in nine HEHE patients from May 2010 to January 2020.ResultThere were 121 lesions in nine patients with a predominantly peripheral distribution. Five lesions (4.13%) in two patients (22.22%) had evidence of capsular retraction, and three patients had lung metastasis (33.33%). Dynamic contrast-enhanced MRI showed progressive enhancement, mainly in two ways: ring enhancement with hypovascularity in four patients (44.44%) and ring enhancement with hypervascularity in five patients (55.56%). Imaging demonstrated a multilayer ring appearance, which was typically observed on T2-weighted imaging (T2WI). The most common appearance consisted of two layers of varying signal, with some images displaying up to four layers. There were significant differences in the size of lesions between different layers of multilayer ring appearance (p < 0.001). All lesions exhibited a two-layer appearance on diffusion-weighted imaging (DWI), with hyperintensity at the periphery and a slightly high signal at the center (except for those with a single layer on T2WI). The “vascular penetration sign” was observed in most lesions, and the blood vessels of 112 lesions (92.56%) were portal vein branches, and five (4.13%) were hepatic vein branches. Pulmonary metastasis was found in three patients with the “vascular penetration sign” of hepatic vein branches.ConclusionThe multilayer ring appearance on T2WI, the “vascular penetration sign”, and the two enhancement patterns may be of great significance in the diagnosis and treatment of HEHE. The “vascular penetration sign” of hepatic vein branches may indicate extrahepatic metastasis.

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Section: Introductionmentioning

confidence: 99%

Novel and Specific MRI Features Indicate the Clinical Features of Patients With Rare Hepatic Tumor Epithelioid Hemangioendothelioma

Zhang

Zhong

et al. 2022

Front. Oncol.

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“…Hepatic EHE has clinicopathological features that are similar to those of angiosarcoma. For that reason, hepatic EHE is sometimes misdiagnosed as angiosarcoma [4]. However, it is important to differentiate hepatic EHE from angiosarcoma because the latter is an aggressive tumor with poor prognosis.…”

Section: Introductionmentioning

confidence: 99%

Hepatic Epithelioid Hemangioendothelioma: Difficult Differential Diagnosis from Angiosarcoma

Taniai

Onda

Sato

et al. 2020

Case Rep Gastroenterol

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Hepatic epithelioid hemangioendothelioma (EHE) is extremely rare, and preoperative diagnosis is difficult because hepatic EHE has clinicopathological features that are similar to those of angiosarcoma. However, it is important to differentiate hepatic EHE from angiosarcoma because the latter is an aggressive tumor with poor prognosis. We herein report a case of hepatic EHE that was difficult to distinguish from angiosarcoma by tumor biopsy. A 30-year-old man with Crohn's disease presented with multiple liver tumors. The tumors were preoperatively diagnosed as angiosarcoma by tumor biopsy. The patient underwent extended left hemihepatectomy with biliary reconstruction and partial resection of segments 6 and 8. Immunohistochemical staining was positive for CD34, factor VIII, and calmodulin binding transcription activator 1 (CAMTA1), and the pathological diagnosis was EHE. Two years after surgery, a recurrent tumor was found in liver segment 6, for which laparoscopic partial hepatectomy was performed. Pathological examination revealed recurrence of EHE. The patient remained well with no evidence of tumor recurrence as of 9 months after the second resection. In conclusion, we

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Primäre Lebersarkome – Empfehlungen zur chirurgischen Therapie

et al. 2022

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Hepatic epithelioid haemangioendothelioma (HEHE): a diagnostic dilemma between haemangioma and angiosarcoma

Cited by 6 publications

References 24 publications

Novel and Specific MRI Features Indicate the Clinical Features of Patients With Rare Hepatic Tumor Epithelioid Hemangioendothelioma

Novel and Specific MRI Features Indicate the Clinical Features of Patients With Rare Hepatic Tumor Epithelioid Hemangioendothelioma

Hepatic Epithelioid Hemangioendothelioma: Difficult Differential Diagnosis from Angiosarcoma

Primäre Lebersarkome – Empfehlungen zur chirurgischen Therapie

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