Primary Hepatic Neoplasms of Vascular Origin: Key Imaging Features and Differential Diagnoses With Radiology-Pathology Correlation

Choi, Hailey H.; Manning, Maria A.; Mehrotra, Anupamjit K.; Wagner, Sean; Jha, Reena

doi:10.2214/ajr.17.18100

Cited by 12 publications

(12 citation statements)

References 48 publications

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“…In contrast, the appearance of a hepatic PEComa on CT or MRI is well defined with early enhancement in the arterial phase and nonuniform enhancement in the venous and delayed phases[ 14 ]. Lesions with delayed washout may mimic HCC[ 15 ]. Consistent with the study results, hepatic PEComa should still be considered whenever a blotchy vascular pattern of the tumor is noted, if there is no evidence of hemorrhage in the tumor, if there is no abnormality in the background parenchyma, when no hepatitis virus markers have been detected, and when liver function tests and tumor markers (AFP, CEA) are normal[ 16 ].…”

Section: Discussionmentioning

confidence: 99%

Perivascular epithelioid cell tumors of the liver misdiagnosed as hepatocellular carcinoma: Three case reports

Kou¹,

Yang²,

Ye³

et al. 2023

World J Clin Cases

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BACKGROUND Hepatic perivascular epithelioid cell neoplasms (PEComas) are rare. Diagnostic and treatment experience with hepatic PEComa remains insufficient. CASE SUMMARY Three hepatic PEComa cases are reported in this paper: One case of primary malignant hepatic PEComa, one case of benign hepatic PEComa, and one case of hepatic PEComa with an ovarian mature cystic teratoma. During preoperative imaging and pathological assessment of intraoperative frozen samples, patients were diagnosed with hepatocellular carcinoma (HCC), while postoperative pathology and immunohistochemistry subsequently revealed hepatic PEComa. Patients with hepatic PEComa which is misdiagnosed as HCC often require a wider surgical resection. It is easy to mistake them for distant metastases of hepatic PEComa and misdiagnosed as HCC, especially when it's combined with tumors in other organs. Three patients eventually underwent partial hepatectomy. After 1-4 years of follow-up, none of the patients experienced recurrence or metastases. CONCLUSION A clear preoperative diagnosis of hepatic PEComa can reduce the scope of resection and prevent unnecessary injuries during surgery.

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Section: Discussionmentioning

confidence: 99%

Perivascular epithelioid cell tumors of the liver misdiagnosed as hepatocellular carcinoma: Three case reports

Kou¹,

Yang²,

Ye³

et al. 2023

World J Clin Cases

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“…Rather than presenting with single, discrete VMs, liver involvement in HHT is typically diffuse and heterogeneous. Histologically, VMs result in both microscopic changes, such as ectatic sinusoids, and macroscopic vascular anomalies [2]. Diffusely distributed hepatic VMs are relatively characteristic of HHT and unusual in other vascular disorders; therefore, diffuse involvement with VMs should always raise suspicion for underlying HHT [8].…”

Section: Hepatic Vascular Malformationsmentioning

confidence: 99%

“…Hereditary hemorrhagic telangiectasia (HHT), also called Osler-Weber-Rendu, is an autosomal dominant disorder characterized by arteriovenous malformations (AVMs) throughout the body [1]. The hallmark lesion of HHT is the telangiectasia, a direct connection between the arteriole and venule, bypassing the capillary bed [2]. HHT most commonly involves mutations of two genes: endoglin (ENG, on chromosome 9, HHT1) or activin A receptor type II-like 1 (ACVRL1/ALK1, on chromosome 12, HHT2).…”

Section: Introductionmentioning

confidence: 99%

“…The Curaçao diagnostic criteria are used to clinically diagnose HHT [9] and are based upon the presence of four findings: (1) spontaneous and recurrent epistaxis, (2) multiple mucocutaneous telangiectases at characteristic sites, (3) visceral involvement, and (4) a first-degree relative with HHT. Depending on the number of criteria that are met, the diagnosis is categorized as "definite" (3 or more criteria), "suspected" (2 criteria), or "unlikely" (1 criterion).…”

Section: Introductionmentioning

confidence: 99%

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The Role of Liver Imaging in Hereditary Hemorrhagic Telangiectasia

Harwin

Sugi

Hetts

et al. 2020

JCM

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Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterized by spontaneous epistaxis, telangiectasia, and visceral vascular malformations. Hepatic vascular malformations are common, though a minority are symptomatic. Symptoms are dependent on the severity and exact type of shunting caused by the hepatic malformation: Arteriosystemic shunting leads to manifestations of high output cardiac failure, and arterioportal shunting leads to portal hypertension. Radiologic imaging, including ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI), is an important tool for assessing liver involvement. Doppler ultrasonography is the first-line screening modality for HHT-related liver disease, and it has a standardized scale. Imaging can determine whether shunting is principally to the hepatic vein or the portal vein, which can be a key determinant of patients’ symptoms. Liver-related complications can be detected, including manifestations of portal hypertension, focal liver masses as well as ischemic cholangiopathy. Ultrasound and MRI also have the ability to quantify blood flow through the liver, which in the future may be used to determine prognosis and direct antiangiogenic therapy.

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“…HHT can involve specific and nonspecific liver lesions in forms ranging from small telangiectasia to large arteriovenous malformations. The hallmark lesion is represented by telangiectasia, a direct connection between the arteriole and venule which bypasses the capillary bed [ 7 ]. The different severities of liver vascular malformation explain the high variability of clinical symptoms [ 6 ].…”

Section: Introductionmentioning

confidence: 99%

Hereditary Hemorrhagic Telangiectasia—A Case Series Experience from a Liver Transplant Center in Romania

et al. 2022

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Hereditary hemorrhagic telangiectasia (HHT) has significant morbidity due to multiorgan involvement and an unpredictable disease course. We analyzed the data of 14 patients diagnosed with HHT. The case series comprised 14 patients with a median age at presentation of 48 years old (41–74 years). In twelve patients (85.7%), the diagnosis was confirmed by using the Curacao Criteria. The most common reason for admission was epistaxis, with 9 patients (57%) presenting with nosebleed refractory to prolonged self-tamponade. The biochemical abnormalities identified were elevations in AP and gamma-GT; liver synthetic function was generally normal, even though 21% of patients had clinical or imaging findings for cirrhosis. Nosebleeds were the main reason for admission and significantly impacted quality of life through anemia and frequent hospital admissions. However, the visceral manifestations seemed to be more serious. The hepatic arteriovenous malformations (AVMs) appeared to remain asymptomatic or led to minimal changes for the majority of patients; some cases were associated with liver and biliary tract ischemia, necrosis leading to acute liver failure and even death. Hepatic AVMs can also lead to high-output heart failure due to arterio-venous shunting. The most frequent AVM was hepatic artery to hepatic vein, with secondary hepatic vein dilation and hemodynamic consequences.

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Primary Hepatic Neoplasms of Vascular Origin: Key Imaging Features and Differential Diagnoses With Radiology-Pathology Correlation

Cited by 12 publications

References 48 publications

Perivascular epithelioid cell tumors of the liver misdiagnosed as hepatocellular carcinoma: Three case reports

Perivascular epithelioid cell tumors of the liver misdiagnosed as hepatocellular carcinoma: Three case reports

The Role of Liver Imaging in Hereditary Hemorrhagic Telangiectasia

Hereditary Hemorrhagic Telangiectasia—A Case Series Experience from a Liver Transplant Center in Romania

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