Hepatic angiomyolipoma is a rare and possibly cancerous mesenchymal tumor that consists of three components: blood vessels, smooth muscle cells, and adipose tissue. In this paper, we reported a case of a 36-year-old man who had a giant hepatic angiomyolipoma with spontaneous rupture and hemorrhage. The patient was admitted to our hospital with sudden upper abdominal pain for 3 h. A giant tumor was found in the left and caudate lobes of the liver, as well as significant blood collection around the liver and in the pelvis. Hemoglobin, liver function test results, and serum tumor maker levels were all within normal ranges. To prevent bleeding, emergency angiography and embolization were performed. During angiography, it was discovered that the tumor was supplied by the left hepatic artery and had a very rich internal blood supply. A massive left hepatic mass of about 11 cm in diameter was found bulging from the surface of the liver and rupturing there during laparoscopic exploration a week later. The rupture was strongly adhered to the smaller curvature of the stomach. The patient underwent laparoscopic left hemihepatectomy and caudate lobectomy, and the tumor specimen was brown, with clear boundaries with the surrounding normal liver parenchyma, and there were a large number of necrotic lesions inside the tumor. Histopathological results confirmed the mass as hepatic angiomyolipoma with negative resection margins. Immunohistochemical staining indicated that the tumor had positive homatropine methylbromide-45. After 13 months of follow-up, no tumor recurrence or metastasis occurred in the patient.

Hepatic angiomyolipoma (HAML) comprises epithelioid angiomyolipoma (EAML) and classic hepatic angiomyolipoma (CAML). The imaging appearance of HAML varies widely, and EAML is more easily misdiagnosed as hepatocellular carcinoma (HCC) than as CAML. The clinical and contrast-enhanced ultrasound (CEUS) features of CAML, EAML and HCC with negative alpha-fetoprotein protein expression (HCC[AFP-]) were retrospectively reviewed. The hyper-vascular type was more commonly found in CAML and EAML lesions than in HCC lesions. Most lesions were hyper-enhanced in the arterial phase. CAMLs showed prolonged hyper-enhancement or iso-enhancement during the portal and late phases on CEUS, making them easily distinguishable from HCC(AFP-). Some EAML lesions (41.7%) were hypo-echoic, similar to HCC(AFP-). However, the hypo-enhancement of EAML lesions occurred later than that of HCC(AFP-) lesions. Thus, our findings may be useful in distinguishing among these lesions to improve diagnostic accuracy.

Introduction: Epithelioid hepatic angiomyolipoma (Epi-HAML) can easily be misdiagnosed as a malignant tumor such as hepatocellular carcinoma (HCC) because of the low-fat content on imaging. We analyzed and compared the magnetic resonance imaging (MRI) features of Epi-HAML and HCC, which would aid in disease diagnosis. Methods: We included 30 pathologically confirmed patients with Epi-HAML and 80 with HCC, who underwent both MRI unenhanced scan and three-phase contrast-enhanced MRI scan. The clinical and MRI features of the two groups were summarized and analyzed. Results: Epi-HAML showed significant differences compared to HCC group in terms of clinical features such as sex preference, age, concomitant diseases (hepatitis B and cirrhosis), and elevated plasma alpha-fetoprotein (AFP) (P < 0.001). In addition, there were statistically significant differences between both tumor types with regard to conventional MRI findings such as a solitary tumor (100 vs. 83.8%, P = 0.018), well-defined (93.3 vs. 71.3%, P = 0.027), mild hyperintensity (40.0 vs. 3.7%, P < 0.001) on DWI with high b-value, fat within the tumor (43.3 vs. 8.8%, P < 0.001), and rare necrosis (3.3 vs. 26.3%, P = 0.016). Besides, Epi-HAML displayed significant differences compared to HCC in terms of contrast-enhanced MRI characteristics such as draining hepatic vein (30.0 vs. 3.8%, P < 0.001), portal vein tumor thrombus (0 vs. 13.8%, P = 0.033), hypointensity at delayed phase (70.0 vs. 95%, P = 0.001), intra-tumor vessel at delayed phase (36.7 vs. 10.0%, P = 0.003), pseudocapsule (20.0 vs. 78.8%, P < 0.001), and prolonged enhancement (56.7 vs. 1.2%, P < 0.001). Conclusion: Epi-HAML frequently occurs in middle-aged women and usually lacks characteristic clinical symptoms. Typically, Epi-HAML presents as an isolated and well-defined tumor with rich vasculature. Specific MRI features such as intra-tumor fat, intra-tumor vessel, draining hepatic vein, prolonged enhancement, and lack of capsule may contribute to a more confident diagnosis of Epi-HAML.

OBJECTIVE: To compare the enhancement pattern of hepatic angiomyolipoma (HAML) on contrast enhanced ultrasound (CEUS) and magnetic resonance (MR).
METHODS: The data of seven patients (females; age 28-52 years; mean, 42 years) with histologically proven HAMLs were retrospectively reviewed. All patients underwent CEUS and MR examination. The images were analyzed by two experienced doctors who blinded to the clinical and pathological information of cases.
RESULTS: The mean diameter of the nodule was 5.7 cm (range: 3.2-10 cm). Histopathologic results revealed 4 nodules to be myomatous type and 3 nodules to be mixed type. All nodules showed hyperenhanced during arterial phase on both CEUS and MRI. During portal and delayed phase, washout was more showed on MRI (5/7, 71.4%) than on CEUS (2/7, 28.6%).
CONCLUSIONS: There is discrepancy of enhancement pattern between CEUS and MRI. The quick wash-in and sustained hyperenhancement on CEUS may be helpful for the diagnosis of HAML.

Hepatic angiomyolipoma (HAML) is a rare and difficult-to-diagnose liver tumour. The aim of this study was to summarize experiences in the management of HAML and to recommend a practical treatment strategy.
We retrospectively studied 92 patients who were diagnosed with HAML and analysed the clinical presentation, histopathological features and treatment of the tumours encountered at our institute from May 2009 to June 2016.
The patients included 67 females and 25 males who underwent at least one radiographic examination. Sixty-eight patients underwent radical hepatectomy, two patients underwent liver biopsy, and 22 patients were treated with radiofrequency ablation after liver biopsy. The tumour cells correspondingly expressed both melanoma cell markers (HMB45, MART-1) and smooth muscle cell markers. Two patients were found to have tumour recurrence (2/92, 2.2%) after radical hepatectomy, and none of the patients died.
Diagnosis of HAML depends on pathological findings. The treatment strategy for HAML should be selected according to the tumour size, liver biopsy, location and clinical symptoms of HAML. Patients should be followed closely after surgery because of the malignant potential of HAML.

To evaluate the diagnostic value of dynamic contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) in the differential diagnosis of hepatic angiomyolipoma (HAML) and hepatocellular carcinoma (HCC) and to clarify the relationship between histopathological features and CT or MRI imaging performances in HAML.
Six HAML and 33 non-cirrhotic HCC patients confirmed by histopathology were retrospectively analyzed. The serum biomarkers, CT and MRI examinations were conventionally performed before the confirmatory histological diagnosis. The clinical data from their medical records was also analyzed.
Six HAML patients were annotated as two types according to CT and MRI imaging characteristics, including hypovascular type (n = 1) and hypervascular type (n = 5). The imaging performances of the 33 HCC patients were hypervascular type. Moreover, all the 5 hypervascular type HAML patients were misdiagnosed as HCC by CT or MRI. We also found that the hypervascular type of HAML patients contained more vessels and less fatty tissues in histopathology than hypovascular type of HAML patients. However, the clinical features included HCC high risk factors (hepatitis B or C), non-specific symptoms, male and increased serum alpha fetoprotein (AFP) were more common in HCC patients than HAML patients (P < 0.05, respectively).
The CT or MRI imaging performances of HAML patients containing more vessels and less fatty tissues in histopathology resemble the imaging performance of HCC patients. These clinical features may be of great help in the differential diagnosis in the current clinical practices.

OBJECTIVE: To compare imaging findings of CT and contrast-enhanced US (CEUS) in hepatic angiomyolipoma (HAML) and investigate their pathological correlations.
METHODS: Imaging findings and preoperative diagnosis of CT and CEUS were retrospectively compared head to head in 46 patients with 54 histologically proven HAMLs. Correlations between imaging features and preoperative diagnosis with pathological types of HAMLs were analyzed.
RESULTS: Fat was detected in 100% of lipomatous type, 84.6% of mixed type, and 7.1% of myomatous type (p = 0.000) of HAML at unenhanced CT. Well-defined hyper-echogenicity was displayed in 100% of lipomatous type, 88.5% of mixed type, 50% of myomatous type, and 66.7% of angiomatous type of HAMLs at unenhanced US. More arterial hyper-enhancement was noted on CEUS (100%) than on CT (73.1%) in mixed type (p = 0.015) and in lipomatous type (90.9% vs. 9.1%, p = 0.000) of HAMLs. Washout was present in more HAMLs on CT than on CEUS (42.6% vs. 18.5%, p = 0.007). Correct preoperative diagnosis was suggested in more HAMLs of myomatous type on CEUS than on CT (42.9% vs. 0%, p = 0.016) but showed no difference in other types of HAMLs.
CONCLUSIONS: There are considerable discrepancies between CT and CEUS findings of HAMLs, and the imaging appearance and preoperative diagnosis of HAMLs on CT and CEUS are significantly affected by pathological types of HAMLs.

Twenty-five pathologically proven hepatic angiomyolipomas (AMLs) were included in the study. Ultrasonic features of hepatic AMLs were reviewed. Three types of echogenicity were observed on ultrasound examination: (i) strong hyper-echogenicity, (ii) moderate hyper-echogenicity and (iii) hypo-echogenicity. Vascular signals within tumors could be detected in 22 (88.00%) tumors as multiple punctiform, filiform or dendriform signals by color Doppler flow imaging. Based on the enhancement patterns in the arterial, portal and late phases, the features of hepatic AMLs on contrast-enhanced ultrasound were divided into four subtypes: (i) \"fast in slow out\" (68.00%, n = 17); (ii) \"fast in same out\" (16%, n = 4); (iii) \"fast in fast out\" (12.00%, n = 3); and (iv) \"fast in uneven out\" (4.00%, n = 1). Contrast-enhanced ultrasound diagnosed 22 (88.00%) tumors as benign tumors and 13 (52.00%) as hepatic AMLs. Four cases were misdiagnosed as hepatic hemangioma, five cases as focal nodular hyperplasia (total = 36.00%). The rate of correct diagnosis of hepatic AMLs increased significantly from 24.00% for ultrasound alone to 52.00% for contrast-enhanced ultrasound. Therefore, information obtained from ultrasound, color Doppler flow imaging and contrast-enhanced ultrasound should be combined to improve diagnosis.

OBJECTIVE: Hepatic angiomyolipoma (AML) is a rare, hepatic mesenchymal neoplasm. Its preoperative diagnosis is very difficult, and the treatment is still controversial. The aim is to summarize experience in diagnosis and management of hepatic AML from a cancer center.
METHODS: We retrospectively reviewed the clinical presentation, histopathological, features and treatment of the tumors encountered at our institute from January 2000 to December 2012.
RESULTS: The patients included six females and two males, with female preponderance. Six patients are asymptomatic. Laboratory tests lack specificity. Combining imaging modality, only one patient obtained the accurate diagnosis of hepatic AML and was confirmed by fine-needle aspiration biopsy combined with homatropine methylbromide-45 staining. All other patients received hepatic resection. There was no tumor recurrence or increase of tumor size within the follow-up period.
CONCLUSIONS: We suggest fine-needle aspiration combined with homatropine methylbromide-45 staining should be performed in all patients who are asymptomatic and without serological abnormalities. Surgical resection might be considered only if the malignant potential of the lesion cannot be ruled out or the tumor size is increasing during observation.