• angiomyolipoma
• genes
• histology
• liver neoplasms
• p53
• perivascular epithelioid cell neoplasms
• prognosis

• Humans
• Perivascular Epithelioid Cell Neoplasms/pathology
• Perivascular Epithelioid Cell Neoplasms/diagnosis
• Female
• Liver Neoplasms/pathology
• Liver Neoplasms/diagnosis
• Male
• Middle Aged
• Adult
• Angiomyolipoma/pathology
• Angiomyolipoma/diagnosis
• Prognosis
• Aged
• Young Adult
• Biomarkers, Tumor/analysis
• Adolescent
• Immunohistochemistry

• Korean government Ministry of Science

• CEUS
• HCC
• Hepatic angiomyolipoma
• Tuberous sclerosis
• Ultrasound-guided biopsy

• AML
• Malignant PEComa
• Malignant transformation
• PEComa
• mTOR inhibitor

• Humans
• Everolimus/therapeutic use
• Male
• Perivascular Epithelioid Cell Neoplasms/pathology
• Perivascular Epithelioid Cell Neoplasms/drug therapy
• Middle Aged
• Kidney Neoplasms/drug therapy
• Kidney Neoplasms/pathology
• Liver Neoplasms/pathology
• Liver Neoplasms/drug therapy
• Disease Progression
• Antineoplastic Agents/therapeutic use
• Neoplasm Recurrence, Local/pathology
• Angiomyolipoma
• Tuberous Sclerosis/complications
• Fatal Outcome

• Angiomyolipomas
• Hepatic perivascular epithelioid cell tumors
• Liver
• Mesenchymal tissue-derived tumors
• Preoperative diagnosis

• Humans
• Perivascular Epithelioid Cell Neoplasms/surgery
• Perivascular Epithelioid Cell Neoplasms/pathology
• Perivascular Epithelioid Cell Neoplasms/diagnosis
• Liver Neoplasms/surgery
• Liver Neoplasms/pathology
• Liver Neoplasms/diagnosis
• Hepatectomy
• Preoperative Care/methods
• Biomarkers, Tumor/analysis
• Diagnosis, Differential
• Liver/pathology
• Liver/surgery
• Liver/diagnostic imaging

• Hepatic epithelioid angiomyolipoma
• TACE
• conservative treatment
• diagnosis
• multiple intrahepatic angiomyolipoma
• regular follow-up

• epithelioid angiomyolipoma
• hepatectomy
• liver tumour
• prognosis

• Angiomyolipoma/diagnostic imaging
• Angiomyolipoma/surgery
• Female
• Humans
• Liver Neoplasms/diagnostic imaging
• Liver Neoplasms/surgery
• Male
• Middle Aged
• Neoplasm Recurrence, Local
• Retrospective Studies

• Diagnosis
• Liver
• Perivascular epithelioid cell neoplasms
• Treatment

Perivascular epithelioid cell neoplasm (PEComa) in a child is very rare. We herein report the first malignant case of PEComa developing in the liver of a pediatric patient.

A 10-year-old boy visited a private clinic with prolonged fever of unknown etiology. Abdominal ultrasonography was performed to evaluate the fever’s origin, revealing a large tumor in the liver. He was thus referred to a nearby hospital to investigate the tumor further. Enhanced computed tomography (CT) showed a 6.8 × 5.9 × 10.5-cm solid lesion on S4 and S5. On magnetic resonance imaging (MRI), the tumor had a low signal intensity on T1 imaging and high signal intensity on T2 imaging, with partial diffusion restriction. ¹⁸F-fluorodeoxyglucose-positron emission tomography (FDG-PET) showed a marked uptake in the mass lesion with no evidence of metastasis. The patient was negative for all tumor markers, including AFP, CEA and PIVKA-II. The results of a needle biopsy suggested hepatocellular carcinoma. The tumor’s rapid growth suggested malignancy. Hepatic segmentectomy (S4 + S5 + S8) was performed. The tumor was resected en bloc with a margin. Microscopically, the tumor showed atypical spindle, polygonal or oval-shaped cells with a high nuclear grade, and vascular invasion. Immunohistochemistry was positive for alpha-smooth muscle antigen (α-SMA), human melanin black-45 (HMB-45) and melan A. The pathological diagnosis was malignant PEComa. In the 6 months after surgery, the patient complained of shoulder pain. MRI showed a dumbbell-shaped tumor at the 2nd thoracic vertebrae, which was confirmed to be bone metastasis of PEComa. After chemotherapy, including ifosfamide and doxorubicin, vertebrectomy was performed. Two years later, thoracoabdominal CT showed a 10-cm solid mass occupying the pelvis and a 15-mm nodule in the middle lobe of the right lung. Under a diagnosis of peritoneal and lung metastases, they were surgically removed and metastasis of PEComa was pathologically confirmed. Four months after the 2nd relapse, pelvic metastasis appeared again and mTOR (mammalian target of rapamycin) inhibitor was initiated. To our knowledge, this is the first report of malignant hepatic PEComa in a pediatric patient.

Although extremely rare, malignant hepatic PEComa can develop in a child.

• 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET)
• Hepatocellular carcinoma
• Perivascular epithelioid cell neoplasm (PEComa)
• Segmentectomy

• Angiomyolipoma
• Imaging
• Liver
• Pathology
• Potentially malignant
• Tuberous sclerosis complex

• Angiomyolipoma/diagnostic imaging
• Angiomyolipoma/surgery
• Carcinoma, Hepatocellular
• Female
• Humans
• Liver Neoplasms/diagnostic imaging
• Liver Neoplasms/therapy
• Middle Aged
• Tomography, X-Ray Computed

• Hepatic angiomyolipoma
• Hepatocellular carcinoma

•

HAML is a rare mesenchymal liver tumour which belongs to the family of perivascular epithelioid cell tumours.

Hepatic angiomyolipoma (HAML) is a rare mesenchymal liver tumour which belongs to the family of perivascular epithelioid cell tumours (PEComas). It is typically composed of blood vessels, smooth muscle, and adipose cells, and shows strong immunoreactivity for HMB-45.

A 57-year-old woman was referred to our hospital with an unclear liver lesion. A fine needle biopsy revealed a suspicion of hepatic angiomyolipoma with extramedullary haematopoiesis. Preoperative imaging revealed a tumour 17 cm in diameter in the left liver lobe segments II and III. A lobectomy of the left lobe segments II and III was performed. The pathological diagnosis of hepatic angiomyolipoma was obtained.

Variations in the predominance of the tissue components in HAML impedes diagnosis based on imaging alone. The most promising evidence of HAML is the histological identification of lipomatous, myomatous, and angiomatous tissue combined with immunohistochemical positivity for HMB-45. Although the tumour is considered benign, some cases have been described with malignant behaviour. Surgical resection should be considered in case of symptoms, inconclusive biopsy, or growth in follow-up. Other surgical indications may include aggressive patterns such as vascular invasion, p53 immunoreactivity, or rapidly proliferating tumour cells.

HAML is a rare liver tumour. In patients with symptoms, uncertain diagnosis, or tumour growth, surgical resection should be performed according to oncological criteria.

• Case report
• HAML
• Hepatic angiomyolipoma
• Hepatic lobectomy
• Liver tumour
• Mesenchymal liver tumor

• Angiomyolipoma, hepatic epithelioid
• Benign liver Neoplasms
• Hemihepatectomy
• Histology
• Ultrasonography, contrast-enhanced

We describe three cases of liver lesions, characterized by a discrepancy between presurgical imaging and histological features, in which the final histological diagnosis was quite different from what the surgeons expected. We present (1) a case of primary liver angiomyolipoma associated with focal nodular hyperplasia, (2) a case of perivascular epithelioid cell tumor, and (3) a case of liver splenosis associated with focal nodular hyperplasia. In all cases, a presurgical diagnosis of hepatocellular adenoma was made. Due to nonspecific clinical and radiological features, these rare liver lesions are often presurgically misdiagnosed, especially in young noncirrhotic patients. The association among different lesions represents one additional diagnostic challenge.

• angiomyolipoma
• focal nodular hyperplasia
• histopathology
• perivascular epithelioid cell tumor
• splenosis

Hepatic angiomyolipoma (AML) is a rare mesenchymal tumour with an undetermined malignant potential. Clinical symptoms are non-specific. The radiological hallmarks are high vascularization of lesion and presence of macroscopic fat. The proportion of fatty tissue varies significantly and discrepancies between pre-operative imaging and histological findings are observed in more than 50% of cases. Visualization of the draining vein may aid in differentiation between AML and hepatocellular carcinoma with abundant fatty component. Biopsy is indicated in ambiguous cases. Presence of clinical symptoms warrants surgical treatment. We present a clinical case of giant hepatic AML, discuss its typical features and treatment options.

• angiomyolipoma
• atypical
• epithelioid
• liver
• resection

• HMB-45
• PEComa
• TFE-3
• differential diagnosis
• liver

• CD68
• hepatic angiomyolipoma
• macrophage

• Angiomyolipoma/diagnosis
• Angiomyolipoma/pathology
• Contrast Media/pharmacology
• Diagnosis, Differential
• Female
• Gadolinium DTPA/pharmacology
• Humans
• Liver Neoplasms/diagnosis
• Liver Neoplasms/pathology
• Macrophages/pathology
• Magnetic Resonance Imaging/methods
• Male
• Middle Aged
• Staining and Labeling

• hepatectomy
• hepatic angiomyolipoma
• liver biopsy
• recurrence

• Hepatic angiomyolipoma
• malignant
• management
• review

• Electron microscopy
• Ethoxybenzyl MRI
• Hepatic angiomyolipoma
• Immunohistochemistry

Hepatic angiomyolipoma is an extremely rare benign hamartomatous lesion. Situs inversus totalis is a genetic condition occurring in 0.01% of the population. Following the kidney, the liver is the second most common site of angiomyolipoma. No consensus on the treatment of hepatic angiomyolipoma has been reached. However, the majority of these tumours are managed conservatively. Situs inversus totalis presents difficulties for procedures and is most commonly an incidental finding. These two conditions have not previously been reported and no genetic link has been established between them. This paper reports the association of both conditions in a 74-year-old female, reviews the literature, and presents CT imaging of the case.

• Angiomyolipoma
• Bile duct adenoma
• Hepatocelluar carcinoam
• Mimicker
• Pseudolymphoma

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.

• Hepatic angiomyolipoma
• Malignant potential
• Perivascular epithelioid cell

• Angiomyolipoma
• Fatty metamorphosis
• Well-differentiated hepatocellular carcinoma

Hepatic angiomyolipomas (AMLs) are typically benign tumors containing varying amounts of smooth muscle cells, adipose tissue, and vessels, and are commonly found in the kidney and occasionally in the liver. The preoperative diagnosis of hepatic AML is primarily made from imaging and fine-needle aspiration biopsy results, though limited experience for such diagnoses can result in misdiagnosis. Some uncommon features of hepatic AML have been reported in the literature without an objective or qualitative consensus. As the majority of cases are benign, conservative treatment of AMLs is recommended. However, in rare cases, liver transplantation has been implemented. Only five cases of malignant hepatic AML have been reported. We report a rare case of recurrent posthepatectomy malignant hepatic AML that was misdiagnosed as liver cancer in a 37-year-old woman, which was treated by liver transplantation. The imaging and pathologic findings are presented in order to provide a more concise description to aid in future diagnoses.

• Angiomyolipoma
• Hepatectomy
• Malignant
• Liver transplantation
• Recurrence

• Adult
• Angiomyolipoma/chemistry
• Angiomyolipoma/pathology
• Angiomyolipoma/surgery
• Biomarkers, Tumor/analysis
• Biopsy
• Female
• Hepatectomy
• Humans
• Immunohistochemistry
• Liver Neoplasms/chemistry
• Liver Neoplasms/pathology
• Liver Neoplasms/surgery
• Liver Transplantation
• Magnetic Resonance Imaging
• Neoplasm Recurrence, Local
• Reoperation
• Time Factors
• Tomography, X-Ray Computed
• Treatment Outcome

The risk of developing hepatocellular carcinoma (HCC) is strongly associated with hepatitis B virus infection. Hepatic angiomyolipoma (AML), a rare benign tumor, is composed of a heterogeneous mixture of adipose cells, smooth muscle cells and blood vessels. Here, we report the case of a 44-year-old man who developed HCC with a concomitant hepatic AML and a cavernous hemangioma, in the absence of cirrhosis. To our knowledge, based on an extensive literature search using the www.pubmed.gov website, this is the first report of an HCC case with both concomitant AML and cavernous hemangioma at the same position in the liver. The presence of the hepatitis B surface antigen was detected, but the liver function was normal. Clinical and pathological data were collected before and during the treatment. Hepatic AML was diagnosed based on the typical histological characteristics and immunohistochemical staining, which revealed, a positive staining with a melanocytic cell-specific monoclonal antibody. There was no evidence of tuberous sclerosis complex in this patient. Although the HCC was poor- to moderately-differentiated, the characteristics of the AML and the cavernous hemangioma in this patient did not match any criteria for malignancy. Hepatectomy followed by transarterial chemoembolization treatment were effective therapeutic methods for the adjacent lesions in this patient. This case is an interesting coincidence.

• Angiomyolipoma
• Hepatocellular carcinoma
• Cavernous hemangioma
• Hepatitis B virus infection
• Concomitant

• Adult
• Angiomyolipoma/chemistry
• Angiomyolipoma/pathology
• Angiomyolipoma/therapy
• Biomarkers, Tumor/analysis
• Biopsy
• Carcinoma, Hepatocellular/chemistry
• Carcinoma, Hepatocellular/pathology
• Carcinoma, Hepatocellular/therapy
• Chemoembolization, Therapeutic
• Hemangioma, Cavernous/chemistry
• Hemangioma, Cavernous/pathology
• Hemangioma, Cavernous/therapy
• Hepatectomy
• Humans
• Immunohistochemistry
• Liver Neoplasms/chemistry
• Liver Neoplasms/pathology
• Liver Neoplasms/therapy
• Male
• Neoplasms, Multiple Primary/chemistry
• Neoplasms, Multiple Primary/pathology
• Neoplasms, Multiple Primary/therapy
• Treatment Outcome
• Ultrasonography, Doppler, Color

• Color Doppler flow imaging
• Contrast-enhanced ultrasound
• Hepatic angiomyolipoma
• Pathology
• Ultrasound

• Adult
• Angiomyolipoma/diagnostic imaging
• Contrast Media
• Diagnosis, Differential
• Female
• Humans
• Image Enhancement
• Liver/diagnostic imaging
• Liver Neoplasms/diagnostic imaging
• Male
• Microbubbles
• Middle Aged
• Phospholipids
• Retrospective Studies
• Sulfur Hexafluoride
• Ultrasonography
• Young Adult

A 51-year-old female had been diagnosed with a hemangioma in the hepatic segment 6 (S6). After a 6-year follow-up, enlargement of the tumor was detected. The tumor was clearly enhanced in the arterial phase, and the enhancement remained in the portal phase on computed tomography (CT). Although the primary differential diagnosis on CT was hepatocellular carcinoma (HCC), we worried about the possibility of other vessel system tumors because the tumor remained to be enhanced at the portal phase for HCC and all tumor markers of HCC were negative. We performed angiography to determine the tumor nature and to seek other tumors. Angiography showed tumor stain at the hepatic S6 with an early obvious drainage vein from the tumor flowing through the right hepatic vein into the inferior vena cava. In addition to tumor stain and the drainage vein, there were many small poolings of contrast medium in the whole liver, which were suspected as dilatation of the hepatic peripheral artery. We suspected the tumor as a benign tumor such as hepatocellular adenoma or focal nodular hyperplasia, but the possibility of HCC could not be ruled out. Hepatic posterior sectionectomy was done to completely remove the drainage vein with the tumor. Intraoperative histological examination revealed the tumor as not malignant and not HCC. Later, immunohistochemical analysis uncovered that the tumor had high expression of HMB-45 and, therefore, the final diagnosis was angiomyolipoma. We think that detecting an early drainage vein from the tumor would be a key point for diagnosing hepatic angiomyolipoma.

• Angiography
• Angiomyolipoma
• Early drainage vein
• Hepatocellular carcinoma
• Liver

Benign liver tumors are common. They do not spread to other areas of the body, and they usually do not pose a serious health risk. In fact, in most cases, benign liver tumors are not diagnosed because patients are asymptomatic. When they are detected, it’s usually because the person has had medical imaging tests, such as an ultrasound (US), computed tomography (CT) scan, or magnetic resonance imaging (MRI), for another condition.

A search of the literature was made using cancer literature and the PubMed, Scopus, and Web of Science (WOS) database for the following keywords: “hepatic benign tumors”, “hepatic cystic tumors”, “polycystic liver disease”, “liver macroregenerative nodules”, “hepatic mesenchymal hamartoma”, “hepatic angiomyolipoma”, “biliary cystadenoma”, and “nodular regenerative hyperplasia”.

Hepatocellular carcinoma (HCC) is one of the most common malignant tumors in some areas of the world; there is an increasing incidence worldwide. Approximately 750,000 new cases are reported per year. More than 75 % of cases occur in the Asia-Pacific region, largely in association with chronic hepatitis B virus (HBV) infection. The incidence of HCC is increasing in the USA and Europe because of the increased incidence of hepatitis C virus (HCV) infection. Unlike the liver HCC, benign tumors are less frequent. However, they represent a chapter always more interesting of liver disease. In fact, a careful differential diagnosis with the forms of malignant tumor is often required in such a way so as to direct the patient to the correct therapy. In conclusion, many of these tumors present with typical features in various imaging studies. On occasions, biopsies are required, and/or surgical removal is needed. In the majority of cases of benign hepatic tumors, no treatment is indicated. The main indication for treatment is the presence of significant clinical symptoms or suspicion of malignancy or fear of malignant transformation.

• angiomyolipoma
• arteriovenous fistula
• diagnostic imaging
• hemodynamics
• liver neoplasm
• surgical pathology

Hepatic angiomyolipoma is a rare benign mesenchymal tumor. We report an unusual case of a patient with multiple hepatic angiomyolipomas exhibiting high ¹⁸ F-fluorodeoxyglucose (FDG) uptake.

A 29-year-old man with a medical history of tuberous sclerosis was admitted to our hospital for fever, vomiting, and weight loss. Abdominal dynamic computed tomography revealed faint hypervascular hepatic tumors in segments 5 (67 mm) and 6 (10 mm), with rapid washout and clear borders; however, the tumors exhibited no definite fatty density. Abdominal magnetic resonance imaging revealed that the hepatic lesions were slightly hypointense on T1-weighted imaging, slightly hyperintense on T2-weighted imaging, and hyperintense with no apparent fat component on diffusion-weighted imaging. FDG-positron emission tomography (PET) imaging revealed high maximum standardized uptake values (SUVmax) of 6.27 (Segment 5) and 3.22 (Segment 6) in the hepatic tumors. A right hepatic lobectomy was performed, and part of the middle hepatic vein was also excised. Histological examination revealed that these tumors were characterized by the background infiltration of numerous inflammatory cells, including spindle-shaped cells, and a resemblance to an inflammatory pseudotumor. Immunohistochemical evaluation revealed that the tumor stained positively for human melanoma black-45. The tumor was therefore considered an inflammatory pseudotumor-like angiomyolipoma. Although several case reports of hepatic angiomyolipoma have been described or reviewed in the literature, only 3 have exhibited high ¹⁸ F-FDG uptake on PET imaging with SUVmax ranging from 3.3–4.0. In this case, increased ¹⁸ F-FDG uptake is more likely to appear, particularly if the inflammation is predominant.

Although literature regarding the role of ¹⁸ F-FDG-PET in hepatic angiomyolipoma diagnosis is limited and the diagnostic value of ¹⁸ F-FDG-PET has not yet been clearly defined, the possibility that hepatic angiomyolipoma might exhibit high ¹⁸ F-FDG uptake should be considered.

AIM: 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.

METHODS: 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.

RESULTS: 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.

CONCLUSION: 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.

• Liver
• Epithelioid
• Angiomyolipoma
• Computed tomography

• Angiomyolipoma/diagnostic imaging
• Angiomyolipoma/surgery
• Contrast Media/chemistry
• Diagnosis, Differential
• Female
• Focal Nodular Hyperplasia/diagnostic imaging
• Humans
• Immunohistochemistry
• Liver/pathology
• Liver Neoplasms/diagnostic imaging
• Liver Neoplasms/surgery
• Male
• Mesoderm/pathology
• Middle Aged
• Postoperative Period
• Radiographic Image Interpretation, Computer-Assisted
• Reproducibility of Results
• Retrospective Studies
• Tomography, Spiral Computed
• Treatment Outcome

• angiomyolipoma
• hepatectomy
• hepatic angiomyolipoma

The purpose of our study was to retrospectively evaluate the computed tomography (CT)/magnetic resonance imaging features of epithelioid angiomyolipoma of the liver (Epi-HAML), with pathology as a reference. We reviewed the CT/MRI findings of six lesions of Epi-HAML and found absence of adipose tissue in the lesions. In addition, recognizing the imaging features of no capsule, and hypervasularity with central punctiform or filiform vessels as a characteristic enhancement may distinguish Epi-HAML from other hepatic tumors.

• Adenoma/diagnostic imaging
• Angiomyolipoma/diagnostic imaging
• Cysts/diagnostic imaging
• Hemangioma/diagnostic imaging
• Humans
• Imaging, Three-Dimensional
• Incidental Findings
• Liver Diseases/diagnostic imaging
• Liver Neoplasms/blood supply
• Liver Neoplasms/diagnostic imaging
• Multidetector Computed Tomography/methods
• Radiographic Image Enhancement/methods
• Telangiectasia, Hereditary Hemorrhagic/diagnostic imaging

• Abdominal Pain/etiology
• Adult
• Angiomyolipoma/pathology
• Angiomyolipoma/surgery
• Biopsy, Fine-Needle
• Female
• Humans
• Immunohistochemistry
• Liver Neoplasms/pathology
• Liver Neoplasms/surgery
• Tomography, X-Ray Computed