Hepatic Epithelioid Angiomyolipoma Research Articles

Hepatic epithelioid angiomyolipoma: magnetic resonance imaging characteristics.

The aim of the study was to analyze MR imaging features of hepatic epithelioid angiomyolipoma (HEAML). This study included 113 patients with 122 pathologically confirmed HEAML who underwent enhanced MRI scanning before partial hepatectomy. MR images were retrospectively reviewed and correlated with pathological findings. The mean age of 113 patients was 48.12 ± 11.77years old, and the male to female ratio was 1:3.35 with 87 females (76.99%). 107 (94.69%) patients presented as single tumor, and 96 patients (84.96%) were asymptomatic. 122 HEAML lesions were diagnosed pathologically in 113 patients, with the average of 4.47 ± 3.26cm. 109 lesions (89.34%) and 108 lesions (88.52%) showed regular and well-defined boundary. On T1WI, 121 lesions (99.18%) mainly presented hypointensity. On T2WI, 118 lesions (96.72%) and 109 lesions (89.34%) mainly showed hyperintensity and heterogeneous signals. Most of the lesions (97.46%) showed hyperintensity on DWI. 118 lesions (96.72%) manifested as severe hyperenhancement and 106 lesions (86.89%) showed heterogeneity during the arterial phase. As for the lesion enhancement pattern, 73 lesions (59.84%) presented as persistent enhancement, 37 lesions (30.33%) as wash out, 8 lesions (6.56%) as degressive enhancement, and 4 lesions (3.28%) as poor blood supply. Additionally, 96 lesions (78.69%) with intra-tumor vessels and 85 lesions (69.67%) with outer rim were confirmed during the delayed phase. At enhanced MRI, hypointensity on T1WI, hyperintensity and heterogeneous signals on T2WI, hyperintensity on DWI, little or no fat component, heterogenous hyperenhancement, persistent enhancement, intra-tumor vessels, and outer rim would be helpful to diagnose HEAML.

Primary hepatic epithelioid angiomyolipoma: a small case series.

Hepatic epithelioid angiomyolipomas (HEAMLs) are rare and usually arise in case reports, and thus, we aimed to study the clinicopathological features, treatment and prognosis of these lesions. We identified patients from institutional database with HEAMLs and retrospectively collected clinical data. Of 12 patients, 10 were females, and the median age was 49 years. A liver mass was found on chance upon routine health screening in nine patients. Imaging features included clear border (9/12), internal heterogeneity (8/12), arterial enhancement (12/12), fat (5/12), intra-tumour vessel (3/12), draining hepatic vein (2/12) and pseudocapsule (1/12). The preoperative diagnoses contained HEAML (n=5), hepatocellular carcinoma (n=6), and hepatic cystadenocarcinoma (n=1). All cases had received surgery and obtained a negative margin. All specimens showed positive findings for HMB-45 and Melan A. No tumour recurrence or mortality was described with a mean follow-up time of 23.5months. HEAML is a low potential malignancy tumour that frequently appears in middle-aged females. It has nonspecific symptoms and may present special imaging features including intra-tumour vessel, early draining hepatic vein and lack of a pseudo capsule. The lesion is confirmed by pathological and immunohistochemical findings. Surgery and subsequent long-term follow-up are the most appropriate management approaches.

Baseline and contrast-enhanced ultrasound features of hepatic epithelioid angiomyolipoma.

Recurrence or metastasis after surgery had been reported in hepatic epithelioid angiomylipoma (epi-AML). Most hepatic epi-AMLs were misdiagnosed with hepatocellular carcinoma or other hepatic tumors before surgery. To describe the baseline and contrast-enhanced ultrasound (CEUS) features of hepatic epi-AMLs and to explore the potential ultrasonic features for prognosis. The retrospective study enrolled 67 patients (56 females, 11 males) with 67 pathologically confirmed hepatic epi-AML lesions. All the lesions were examined by baseline ultrasound and 42 lesions were examined using CEUS with SonoVue (Bracco, Milan, Italy) before surgery. Baseline ultrasound features of hepatic epi-AMLs included heterogeneous echo (86.6%), well-defined border (68.7%), hypoecho (64.2%), regular morphology (62.7%), peripheral-tumor arc-shaped or ring-like vessels (53.7%), and low value of resistive index (0.51±0.08). CEUS features of hepatic epi-AMLs included arterial phase hyper-enhancement with smooth and well-defined margin (100%), peripheral-tumor ring-like vessels (57.1%), and intra-tumor vessels (52.4%). Some CEUS features, including arterial phase heterogeneously tortuous filling, intra-tumor vessels and peripheral-tumor ring-like vessels were more commonly found in hepatic epi-AMLs of uncertain malignant potential/malignant than in benign hepatic epi-AMLs (p < 0.05). Baseline ultrasound and CEUS features may be useful in diagnosis of hepatic epi-AML, and some CEUS features may be indicative of its malignant potential.

Ultrasound contrast agent Sonazoid for the diagnosis of hepatic epithelioid angiomyolipoma: a case report

BackgroundAn angiomyolipoma usually occurs in the kidneys and rarely in the liver. Hepatic epithelioid angiomyolipoma (HEAML), a rare variant of angiomyolipoma, possesses malignant potential and mimics the imaging features of hepatocellular carcinoma. Sonazoid® (perfluorobutane microbubbles), a new contrast agent that facilitates hepatic parenchyma-specific Kupffer phase imaging on contrast-enhanced ultrasonography (CEUS), is useful for the detection and characterization of focal liver lesions.Case presentationA 30-year-old man with HEAML underwent CEUS using Sonazoid®, in which new concepts for ultrasonography-based differentiation between HEAML and hepatocellular carcinoma were applied.ConclusionsThis case report provides clinicians and radiologists with a reference for distinguishing HEAML from hepatocellular carcinoma based on CEUS using Sonazoid®.

Differentiating Hepatic Epithelioid Angiomyolipoma From Hepatocellular Carcinoma and Focal Nodular Hyperplasia via Radiomics Models.

Background: We conduct a study in developing and validating two radiomics-based models to preoperatively distinguish hepatic epithelioid angiomyolipoma (HEAML) from hepatic carcinoma (HCC) as well as focal nodular hyperplasia (FNH).Methods: Totally, preoperative contrast-enhanced computed tomography (CT) data of 170 patients and preoperative contrast-enhanced magnetic resonance imaging (MRI) data of 137 patients were enrolled in this study. Quantitative texture features and wavelet features were extracted from the regions of interest (ROIs) of each patient imaging data. Then two radiomics signatures were constructed based on CT and MRI radiomics features, respectively, using the random forest (RF) algorithm. By integrating radiomics signatures with clinical characteristics, two radiomics-based fusion models were established through multivariate linear regression and 10-fold cross-validation. Finally, two diagnostic nomograms were built to facilitate the clinical application of the fusion models.Results: The radiomics signatures based on the RF algorithm achieved the optimal predictive performance in both CT and MRI data. The area under the receiver operating characteristic curves (AUCs) reached 0.996, 0.879, 0.999, and 0.925 for the training as well as test cohort from CT and MRI data, respectively. Then, two fusion models simultaneously integrated clinical characteristics achieved average AUCs of 0.966 (CT data) and 0.971 (MRI data) with 10-fold cross-validation. Through decision curve analysis, the fusion models were proved to be excellent models to distinguish HEAML from HCC and FNH in comparison between the clinical models and radiomics signatures.Conclusions: Two radiomics-based models derived from CT and MRI images, respectively, performed well in distinguishing HEAML from HCC and FNH and might be potential diagnostic tools to formulate individualized treatment strategies.

Hepatic nodules with arterial phase hyperenhancement and washout on enhanced computed tomography/magnetic resonance imaging: how to avoid pitfalls

This essay aimed to illustrate the various hepatic nodules that may exhibit arterial phase hyperenhancement and washout on computed tomography/magnetic resonance imaging (CT/MRI). Hepatic nodules with arterial phase hyperenhancement and washout on CT/MRI include hepatocellular carcinoma, focal nodular hyperplasia-like nodules, serum amyloid A-positive hepatocellular neoplasms, intrahepatic cholangiocarcinoma, intrahepatic bile duct adenoma,hepatoblastoma, hepatocellular adenoma, hepatic epithelioid angiomyolipoma, and metastasis including neuroendocrine and gastrointestinal stromal tumors. Understanding the imaging findings is important to ensure correct diagnosis.

Comparison of hepatic epithelioid angiomyolipoma and non-hepatitis B, non-hepatitis C hepatocellular carcinoma on contrast-enhanced ultrasound

PurposeThe purpose of this study was to retrospectively compare the imaging features of hepatic epithelioid angiomyolipoma (HEAML) to those of hepatocellular carcinoma negative for hepatitis B surface antigen and hepatitis C antibody (NBNC-HCC) on contrast-enhanced ultrasound (CEUS) with sulphur hexafluoride microbubbles. Material and methodsTwenty-two patients (4 men, 18 women) with a mean age of 42.6±10.2 (SD) years (range: 22–63 years) with histopathologically confirmed HEMAL were included in the study. Forty-four patients (30 men, 14 women) with a mean age of 57.3±15.9 years (range: 19-85 years) with histopathologically confirmed NBNC-HCC were randomly selected from our institution's database as a control group. The CEUS characteristics of the two groups were compared. ResultsOn conventional ultrasound, significant differences in tumor diameter were found between HEAML (4.0±2.0 [SD] cm; range: 1.3–8.9cm) and NBNC-HCC (8.4±4.4 [SD] cm; range: 1.6-18cm) (P<0.001) as well as in degrees of enhancement during the portal (P=0.001) and late phases (P=0.003), contrast distribution (P<0.001) and absence of pseudocaspule (P<0.001). On CEUS, hyperenhancement during the arterial phase was observed in 21/22 (95.5%) HEAMLs and in 43/44 (97.7%) NBNC-HCCs (P>0.999). Homogeneous enhancement was more frequent in HEAMLs (20/22; 90.9%) than in NBNC-HCCs (13/44; 29.6%) (P<0.001). Pseudocapsule was observed in 0/22 HEAMLs (0.0%) and in 36/44 NBNC-HCCs (81.8%) (P=0.017). A prolonged enhancement was observed in 5/22 HEAMLs (22.7%) and in 0/44 NBNC-HCCs (0.0%) (P<0.001) during the late phase. ConclusionCEUS with sulphur hexafluoride microbubbles is helpful in discriminating between HEAML and NBNC-HCC. Homogeneous enhancement and lack of pseudocapsule are suggestive features for the diagnosis of HEAML.

Contrast-Enhanced Ultrasound Findings and Differential Diagnosis of Hepatic Epithelioid Angiomyolipoma Compared with Hepatocellular Carcinoma

Hepatic epithelioid angiomyolipoma (EAML) has malignant potential that can easily be misdiagnosed as hepatocellular carcinoma (HCC), but the treatment options of these conditions are different. This retrospective study investigated whether contrast-enhanced ultrasound (CEUS) can differentiate EAML from HCC. We included 24 patients with pathologically confirmed EAML and 36 patients with HCC who underwent pre-operative conventional ultrasound and CEUS. The conventional ultrasound and CEUS features of the two groups were analyzed. Time intensity curves (TICs) were analyzed for all lesions using quantitative perfusion analysis software. This study found that significant differences existed between the EAML and HCC groups in terms of clinical features such as sex, age, symptoms, alpha-fetoprotein and history of hepatitis B virus infection (p < 0.05). Tumor size, number, border and echogenicity on conventional ultrasound were significantly different between EAML and HCC (p < 0.05). Regarding CEUS, EAML was more likely to have a centripetal enhancement pattern than HCC (p < 0.05). The tumor enhancement degree in the delayed phase in CEUS was significantly different between EAML and HCC (p < 0.05). Quantitative analysis of CEUS parameters revealed that the wash-in area under the curve (AUC) was larger in EAML than in HCC (p < 0.05). Time to peak was significantly shorter in EAML than in HCC (p < 0.05). The peak intensity was significantly stronger in EAML than in HCC (p < 0.05). Regarding the diagnostic performance of CEUS parameters that showed statistical significance on univariate analysis, the sensitivity and specificity for distinguishing EAML from HCC was 66.7%–100.0% and 55.6%–91.7%, respectively. Overall, CEUS could be valuable in differentiating EAML from HCC. Specific features such as the centripetal filling and prolonged enhancement patterns on CEUS, higher peak intensity, bigger wash-in AUC and shorter time to peak on TICs may contribute to a more confirmative differential diagnosis of EAML.

409 patients with hepatic epithelioid angiomyolipoma: A pooled analysis

Objective To summarize our experience in the diagnosis and treatment of hepatic epithelioid angiomyolipoma (HEAML), with the aim to reduce the future misdiagnosis rate. Methods The PubMed, Medline, China Science Periodical Database (CSPD), and VIP Databases were searched from January 2000 to March 2018 on all reports on HEAML. Results There were 409 cases of HEAML in 97 reports. The ratio of men to women was 1:4.84. The age ranged from 12 to 80 years and the median age was 44 years. 61.9% of patients (205/331) were asymptomatic, while 34.7% (115/331) had upper or right upper quadrant abdominal discomfort. Some patients presented with abdominal mass, gastrointestinal reaction, low grade fever or weight loss. The clinical symptoms in 78 patients were not mentioned in the reports. The misdiagnostic rate of HEAML was as high as 40.3% (165/409). The imaging findings of HEAML were non-specific. Ultrasound, CT and MRI scan usually showed contrast enhancement in the arterial phase. Most lesions were accompanied by central vessels with early drainage veins. The enhanced scans showed varied characteristics. The ratios of fast wash-in and fast wash-out, to fast wash-in and slow wash-out, and to delayed enhancement were roughly 4:5:1. A definitive diagnosis of HEAML is based on the pathological findings of epithelioid cells in the lesions and the expressions of HMB45, SMA, Melan-A and Actin on immunohistochemical staining. HEAML had a relatively low malignant rate of 3.9%. Surgical resection was the main treatment for HEAML. Conclusion HEAML was a rare and easily misdiagnosed disease. ,which could be diagnosed by taking into account the clinical course, imaging, pathological and immunohistochemical findings.HEAML Key words: Hepatic epithelioid angiomyolipoma (HEAML); Imaging; Pathology; Immunohistochemistry; Misdiagnosis

CT features of hepatic epithelioid angiomyolipoma: differentiation from hepatocellular carcinoma in patients with noncirrhotic livers.

Hepatic epithelioid angiomyolipoma (HEA) shares some similarities with other hepatic tumors, such as hepatocellular carcinoma (HCC). Thus, establishing a definite diagnosis of HEA based on medical imaging is often difficult. In this study, we evaluated multiphasic computed tomography (CT) imaging to differentiate HEA from HCC in patients with noncirrhotic livers. We retrospectively analyzed the clinical and imaging data of both contrast and non-contrast enhanced CT scans from 25 patients with HEA and 50 patients with HCC in noncirrhotic livers. CT features, including lesion position, size, shape, interior content, border, enhancement degree, and enhancement pattern, were independently evaluated by two radiologists. Intratumoral blood vessels, peripheral supply vessels, the early display of the hepatic vein, peripheral abnormal perfusion, peripheral washout sign, pseudocapsule, and portal tumor thrombus were also evaluated. Next, we quantitatively analyzed difference within results of clinical and CT characteristics between the HEA and HCC groups. The number of female HEA patients is more than male (76% vs. 24%), with a mean age of 49.44±10.33 years (from 30 to 68 years). The majority (64%) of HEA patients were asymptomatic, without hepatitis (88%). On non-contrast enhanced CT, HEA mainly manifested as a round (92%), hypodense mass (100%) with little fat (12%) and rare complications, such as hemorrhage (4%) and calcification (4%). HEA all manifested as an intensely enhanced mass on contrast-enhanced CT. The differences between HEA and HCC were significant in the imaging characteristics of the early display of the hepatic vein (32% vs. 0%, P=0.000), intratumoral blood vessels during the nonarterial phase (36% vs. 8%, P=0.003), washout enhancement (52% vs. 86%, P=0.001), and prolonged enhancement (40% vs. 4%, P=0.000). Although HEA is an uncommon hepatic tumor, clinical and CT manifestation may be indicative. Clinical and CT characteristics including asymptomatic, non-hepatic, fat-deficient, early display of the hepatic vein, intratumoral blood vessels during the nonarterial phase and prolonged enhancement are selected to improve the recognition of HEA, supporting for a differential diagnosis from HCC.

Malignant potential of epithelioid angiomyolipomas of the liver: A case report and comprehensive review of the literature.

Hepatic epithelioid angiomyolipoma (HEAML) is a rare mesenchymal tumor that has been reported to have malignant potential. We herein describe a rare case of atypical HEAML. A 43-year-old Caucasian male patient visited his general practitioner due to a productive cough persisting for >2 months. During evaluation with several imaging tests, a chest computed tomography (CT) incidentally revealed a well-demarcated lesion in the caudate lobe of the liver, sized 7 cm. An abdominal magnetic resonance imaging examination confirmed the findings of the CT. The possibility of hepatocellular carcinoma at that time could not be excluded. Due to inconclusive cross-sectional imaging, the patient underwent left hepatectomy with additional resection of segment I. The patient's postoperative course was uneventful. A diagnosis of 'atypical' HEAML was established in the present case. The majority of HEAMLs are considered to be benign, although there are several reported cases exhibiting malignant behavior, such as tumor growth, presence of atypical cells, recurrence after surgical resection, metastasis and invasive growth into the liver parenchyma and alongside the vessels. From 2000 onwards, 19 cases of malignant hepatic AML have been reported. Malignant transformation is considered to occur mostly in the epithelioid subtype. To that end, when epithelioid or atypical characteristics are identified on preoperative biopsy, resection is indicated due to the high probability of malignancy.

Imaging features of hepatic epithelioid angiomyolipomas on real-time contrast-enhanced ultrasound

Objective To evaluate the imaging features of hepatic epithelioid angiomyolipoma (EAML) on contrast-enhanced ultrasound (CEUS). Methods The imaging features of pathologically proved hepatic EAML lesions in 17 patients who had undergone baseline ultrasound and CEUS examinations were evaluated retrospectively. Results All of the cases were single lesion. 70.6% (12/17) of the lesions were hypoechoic on ultrasound and 82.4% (14/17) of the lesions were heterogeneous. Flow signals were detected in 88.2% (15/17) of the lesions and 82.4%(14/17) of the lesions showed ringlike or arc arterials peripherally on color Doppler flow imaging (CDFI). The value of RI was 0.38-0.56. On CEUS, all lesions exhibited remarkable hyperenhancement in the arterial phase. 29.4% (5/17) of the lesions showed branched enhancement, 70.6% (12/17) of the lesions showed diffusely entire enhancement. Strong ring-like enhancement peripherally was detected in 29.4% (5/17) of the lesions. 88.2% (15/17) of the lesions were detected homogeneously enhancement during the peak period on CEUS. Washout of contrast agent and hypoechogenicity to surrounding liver tissue during portal or late phase were observed in 41.2% (7/17) and 64.7% (11/17) of the cases retrospectively. Conclusions The combination of real-time CEUS and baseline US can improve the correct diagnosis of hepatic EAML. Key words: Contrast-enhanced ultrasound; Hepatic tumors; Angiomyolipoma, Epithelioid

Hepatic epithelioid angiomyolipoma with prominent invasive growth pattern: a hitherto unreported histopathologic feature which might promote misdiagnosis

Hepatic epithelioid angiomyolipoma with prominent invasive growth pattern: a hitherto unreported histopathologic feature which might promote misdiagnosis

Hepatic epithelioid angiomyolipoma: clinical features and imaging findings of contrast-enhanced ultrasound and CT

To evaluate the clinical features and imaging findings of epithelioid angiomyolipoma (EAML) on contrast-enhanced ultrasound (CEUS) and contrast-enhanced computed tomography (CECT). During January 2006 to November 2015, six patients with histopathologically proven hepatic EAML were evaluated using both CECT and ultrasonography. Six patients underwent CEUS and four patients had CECT images. Patients' demographics, clinical presentations, laboratory test results, and imaging appearances were collected and analysed. Six patients were female, and the mean age was 43.5 years. All hepatic EAMLs were solitary. None of patients had specific symptoms, and laboratory tests were normal in all patients. The maximal diameter of the lesions ranged from 3.1 to 6.5cm (mean 4.4cm). Baseline ultrasound revealed that 50% of hepatic EAMLs were hypo-echoic and 50% were mixed echoic. CEUS showed that 100% of the hepatic EAMLs displayed homogeneous hyperenhancement in the arterial phase and 67% (4/6) showed hypo-enhancement in the late phase. On CECT, 100% (4/4) of the neoplasms were significantly hyperenhanced in the arterial phase and washout in the portal phase. There was no recurrence or metastasis after a mean follow-up period of 11.8 months. Hepatic EAML shows similar enhancement patterns to that of hepatocellular carcinoma. It should be considered as a possible differential diagnosis in female patients without viral hepatitis infection.

Hepatic epithelioid angiomyolipoma is a rare and potentially severe but treatable tumor: A report of three cases and review of the literature.

Hepatic epithelioid angiomyolipoma (EAML) is a rare type of hepatic tumor. Due to a lack of adequate understanding about this tumor, hepatic EAML is often misdiagnosed as other diseases with similar clinical characteristics such as hepatic cancer. In the present study, 3 cases of hepatic EAML are reported, and the main clinicopathological features of this disease are presented, based on a literature search that included articles published in English between February 2000 and September 2014. A total of 24 hepatic EAML cases were considered, of which, 17 were females and 4 presented multiple liver lesions. Among the patients with single lesions, 2 underwent surgery and relapsed after 5 months and 9 years, respectively. Immunohistochemical staining was positive for human melanoma black-45 in the present 3 cases. The aim of the present study was to focus the attention of clinicians on this type of hepatic tumor in order to improve its diagnosis and treatment.

Malignant hepatic epithelioid angiomyolipoma with recurrence in the lung 7years after hepatectomy: a case report and literature review.

Angiomyolipoma (AML) arising in the liver is rare and usually benign, but it occasionally has malignant potential. A 58-year-old man with a liver tumor identified by a previous doctor with features suggestive of hepatocellular carcinoma on computed tomography (CT) underwent anterior segmentectomy of the liver in 2006. Microscopically, the tumor was composed of exclusively epithelioid cells that were scatteredly positive for human melanoma black 45 on immunohistochemistry. Accordingly, primary hepatic epithelioid AML (eAML) was diagnosed. The patient was subsequently referred to our hospital for follow-up after hepatectomy. He had event-free survival for nearly 7 years. In 2013, two well-defined round nodules were detected in the right lung field by chest CT, and partial pneumonectomy was performed for diagnosis and treatment. Histological examination of the resected lung tissue showed that it was morphologically and immunohistochemically identical to his primary hepatic eAML, leading to the diagnosis of pulmonary metastasis. This paper demonstrates a rare case of malignant hepatic eAML with late recurrence in the lung after hepatectomy.

Primary hepatic epithelioid angiomyolipoma: A malignant potential tumor which should be recognized.

To improve the clinical diagnosis and recognition of hepatic epithelioid angiomyolipoma (HEAML). Four cases of primary HEAML were confirmed based on the pathology archive system in our hospital from January 2009 to November 2015. The general state, clinical symptoms, imaging manifestations, histological results and immunohistochemistry of these patients were retrospectively reviewed and analyzed. Studies of HEAML published in the last 15 years were collected from PubMed and MEDLINE to summarize the clinical symptoms, imaging characteristics, pathological features and management of HEAML. Four cases of primary HEAML were retrieved from our archives. These included three female patients and one male patient, with a mean age of 41.8 ± 11.5 years (ranging from 31 to 56 years). The mean tumor size was 7.3 ± 5.5 cm (ranging from 3.0 to 15 cm). In the contrast-enhanced imaging, the tumor was obviously enhanced in the arterial phase, but enhanced continuously or exhibited a slow-density masse during the venous and delayed phases. Histologically, the tumors mainly consisted of epithelioid cells that comprised approximately 95% of the total neoplastic mass. Although no metastases occurred in our patients, pathological studies revealed necrosis, mitotic figures and liver invasion in two patients, which indicates aggressive behavior. Immunohistochemical staining revealed that human melanoma black 45 (HMB-45) and Melan-A were positive in 4 cases. We only identified 81 cases with primary HEAML, including our present patients, from 26 articles available from PubMed and MEDLINE. The majority of the papers were published as case reports. Only 5 (5/75, 6%) cases were associated with tuberous sclerosis complex (TSC). More than half (35/66) were discovered incidentally upon physical examination. Approximately 65% (22/34) of the patients were misdiagnosed with HCC or other tumors before surgery. Approximately 10% (8/81) of the patients with HEAML had recurrence or metastasis after surgery, which was a very high and alarming rate. HEAML is a very rare primary hepatic tumor that is often misdiagnosed before surgery. Patients should be followed closely after surgery because of its malignant potential.

Gd-EOB-DTPA-enhanced and diffusion-weighted magnetic resonance findings in hepatic epithelioid angiomyolipoma: A case report.

The present study reports a case of histologically proven hepatic epithelioid angiomyolipoma that was evaluated with gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid (Gd-EOB-DTPA)-enhanced magnetic resonance imaging and diffusion-weighted imaging. A 23-year-old female was admitted to the Northern Jiangsu People's Hospital (Yangzhou, Jiangsu, China) due to a 5.6-cm mass in the liver, and a right partial hepatectomy was performed. Magnetic resonance imaging (MRI) revealed a hypointense mass on T1-weighted imaging, and a hyperintense mass on T2-weighted and diffusion-weighted imaging, with a higher apparent diffusion coefficient value compared with normal liver parenchyma. On the dynamic Gd-EOB-DTPA-enhanced MRI scan, the lesion manifested as hypervascular with multiple filiform vessels and a pseudocapsule image, and in the hepatobiliary phase the lesion demonstrated a lack of contrast retention, thus appearing hypointense compared with the background liver. Pre-operatively, EMAL was diagnosed on the basis of these findings in the tumor. The optimum treatment is complete surgical excision and subsequent follow-up. The patient was healthy and free from recurrence at 6 months and 1 year post-surgery. Therefore, knowledge of EAML specific features on dynamic Gd-EOB-DTPA-enhanced and DWI scans may improve the diagnostic accuracy of hypervascular hepatic tumors and may facilitate treatment selection.

Hepatic epithelioid angiomyolipoma: a clinicopathologic analysis of 25 cases

To study the clinicopathologic features, immunophenotype, histological diagnosis and prognosis of hepatic epithelioid angiomyolipoma. Clinical data of 25 cases of hepatic epithelioid angiomyolipoma were collected along with follow-up study of the patients. The pathological features were documented and immunohistochemical study of various markers was performed with an emphasis on diagnosis and differential diagnosis. Hepatic epithelioid angiomyolipoma was more commonly found in young women without characteristic clinical symptoms. Its morphological features were characterized by marked cytological atypia, relatively rare mitotic figures; radial distribution of tumor cells around the thin-walled blood vessels or muscular vessels; and the presence of common multinucleated giant cells and large ganglion-like tumor cells. The tumor cells expressed both melanoma cell markers (HMB45, MART-1) and smooth muscle cell markers (SMA). Tumor cells expressed various other markers including ER 16% (4/25), PR 32% (8/25), TFE3 24% (6/25) and p53 60% (15/25). Hepatic epithelioid angiomyolipoma has variable morphological features and characteristic immunohistochemical phenotype. The differential diagnoses include a variety of tumors. The biological behavior of the tumor tends to be benign.

Multi-slice computed tomography manifestations of hepatic epithelioid angiomyolipoma

To explore the characteristics of multi-slice computed tomography (CT) manifestations of hepatic epithelioid angiomyolipoma (HEA), improve the rate of accurate diagnosis, and reduce the misdiagnostic rate. The multi-slice CT manifestations in five patients who were diagnosed with HEA definitely by postoperative pathological examination were analysed retrospectively. Three female patients and two male patients were included. Before operation, four patients received plain CT scanning and dynamic enhancement scanning, and the other patient only received enhancement scanning, with immunohistochemical analysis conducted after postoperative pathological examination. Four patients were misdiagnosed by CT, including three patients misdiagnosed with hepatic cell carcinoma and one patient with focal nodular hyperplasia. Upper abdominal multi-slice spiral CT scanning and three-stage enhancement scanning were conducted in five patients with HEA before operation. HEA had certain characteristic CT manifestations: low density masses, a few relatively high-density masses or fat-density masses diffusely shown in foci, clear boundary, round or oval and large focus, and tumour size ranging from 3.1 cm × 2.5 cm to 7.0 cm × 5.2 cm. During enhancement scanning, the foci were significantly enhanced uniformly or non-uniformly during the arterial phase, while during the venous and equilibrium phases, the foci were enhanced continuously or showed obvious low-density masses. Obviously enhanced and widened vessels could be found adjacent to foci or in the central area of foci during the arterial phase. CT manifestations of HEA have certain characteristics. Primary diagnosis can be obtained by combining CT findings with clinical data, but pathological examination is still needed for a definite diagnosis.