BACKGROUND: Hepatocellular adenoma is a rare benign liver tumor. Typically, hepatocellular adenomas are solitary and are found in young women who use estrogen-containing contraceptives. The occurrence of multiple hepatocellular adenoma has been linked to higher body mass index, and as the prevalence of overweight increases, multiple hepatocellular adenomas are seen more often. An hepatocellular adenoma does not always necessitate treatment, as they can regress under conservative strategies. In incidental cases, an adenoma presents owing to bleeding, which is mostly self-limiting. If it is not, embolization of hepatic involved vessels is indicated.
METHODS: In this case report, we discuss a 42-year old Caucasian woman with multiple hepatocellular bleeds, treated by multiple endovascular procedures. After the first embolization of an adenoma in the right liver lobe, a second bleed occurred in the left lobe, necessitating additional endovascular intervention. During admittance, treatment was complicated by pulmonary embolism and a pneumonia. During follow-up, our patient was diagnosed with antiphospholipid syndrome.
CONCLUSIONS: Hepatocellular adenoma is a rare diagnosis that requires centralized expertise. This particular case illustrates the complexity of treatment strategies for associated intra-abdominal bleeding and possible complications. Although liver adenoma is often an incidental finding, it can also result in significant morbidity. Centralization of treatment leads to expertise in managing complex treatment strategies.

BACKGROUND: pulmonary arterial hypertension (PAH) is a rare complication of hepatic diseases with portal hypertension that, however, has a significant influence on prognosis. We present a mini-review of how to diagnose and treat it based on a clinical case.
METHODS: in early childhood, a patient had portal hypertension associated with cavernous transformation of the portal vein. It was successfully treated by reno-splenic surgery. At the age of 20 years, this patient experienced increased dyspnea at minimal physical activity after the hepatic biopsy due to a hepatocellular adenoma. The examination in the specialized unit showed PAH, which was evaluated as associated with portal hypertension (PAH-PoH). The specific two-drug combination therapy was started with prominent improvement in patient\'s state. Successful surgical tumor treatment was provided some months later. The practical and clinical approaches to the diagnosis and treatment of PAH-PoH are discussed. It was emphasized that not all patients with portal hypertension have pulmonary hypertension, which needs to be treated. A lot of evidence gaps exist in management of these patients.
CONCLUSIONS: all patients, even with past history of portal hypertension, should be monitored closely and screened for PAH earlier, for better results of treatment.

Hepatocellular adenomas (HCAs) are rare benign tumors of the liver that occur predominantly in women taking oral contraceptives. In children, HCAs comprise < 5% of hepatic tumors. We report a case of HCAs in a 7-year-old girl with estrogen and glucose imbalance.
A 7-year-old girl was presented to our hospital with bilateral breast enlargement for 2 months, polydipsia, polyuria, polyphagia, hyperglycemia, and significant weight gain. Computed tomography (CT) showed a 7.2 cm×6.9 cm×5.3 cm round-shaped mass in the left inner lobe of the liver, ovarian ultrasound showed multiple follicles in the ovaries bilaterally, and cranial magnetic resonance imaging (MRI) showed an enlarged superior pituitary. Hematological and biochemical results were as follows: fasting glucose was 19.7 mmol/L, estradiol was 122.9 pmol/L, follicle-stimulating hormone 10.81 IU/L, luteinizing hormone 10.99 IU/L, insulin-like growth factor 1,513 ng/mL, glutamine aminotransferase 86 U/L, and alkaline phosphatase 362 U/L. Thyroid functions, methemoglobin, fetal protein, carcinoembryonic antigen, and chorionic gonadotropin were normal. The patient had a complete surgical resection of the liver tumor, and the postoperative histopathological diagnosis was HCAs. After the surgery, insulin was injected and the glucose levels were stable. During the 36-month follow-up period, neither tumor recurrence nor significant abnormalities were detected using color Doppler ultrasound of the liver. The child\'s precocious puberty is currently under control.
HCAs are particularly rare in children with liver tumors, and risk factors for the development of HCAs in children include sex hormone imbalance, obesity, Fanconi anemia (FA), glycogen storage diseases (GSDs) type I, III, and IV, galactosemia, immunodeficiency, congenital portosystemic shunts (CPSS), cardiac hepatopathy status-post Fontan procedure, Hurler syndrome, familial adenomatous polyposis, germline HNF1A mutations, and maturity-onset diabetes of the young type 3. Most HCAs are detected during a physical examination without clinical symptoms, and some patients may present with symptoms such as abdominal pain, abdominal distension, and abdominal masse. Serum liver function tests can show increased alkaline phosphatase (ALP) and γ- glutamyl transferase (GT), whereas α-Fetoprofein (AFP) levels are normal. The definitive diagnosis relies mainly on histopathological examination. Because HCAs can rupture and bleed and become malignant. Early surgical treatment is recommended after detection.

Hepatic adenomatosis is a rare disease consisting of multiple adenomas in otherwise-normal liver parenchyma. Though the discovery of this entity goes back several years, its diagnosis is still challenging in terms of its definition and pathophysiology. Clinically, patients may be completely asymptomatic and the diagnosis is only made incidentally through imaging tests. The discovery could be made when complications occur such as intraperitoneal hemorrhage with hypovolemic shock due to the rupture of an adenoma. We report a fatal case of a ruptured adenoma in a case of hepatic adenomatosis discovered at autopsy. In order to achieve a better view of this disease, we conducted a literature review on this subject describing the pathogenesis, manifestations, and autopsy contribution to addressing this entity.

Hepatocellular adenomas are rare diseases, defined as benign liver neoplasms composed of cells with hepatocellular differentiation. Differential diagnosis of hepatocellular adenoma from other lesions, including focal nodular hyperplasia and hepatocellular carcinoma, is crucial to determine treatment strategy. We describe a case of β-catenin-activated inflammatory hepatocellular adenoma with malignant transformation. A 50-year-old man with a suspected liver tumor, based on abdominal ultrasonography findings, was referred to our hospital. Contrast-enhanced computed tomography and magnetic resonance imaging revealed a liver tumor in S2 which was enhanced in the arterial phase to the delayed phase. Based on diagnostic imaging findings, hepatocellular adenoma or focal nodular hyperplasia was suspected. We considered the possibility of malignant potential because of the enlargement of the lesion. Thus, we performed a laparoscopic hepatectomy. Histological examination showed pigment deposition in the hepatocytes, which was determined to be lipofuscin. Mild nuclear swelling and atypia in the tumor area indicated nodular growth. Based on the histological and immunohistochemical findings, the diagnosis was ꞵ-catenin-activated inflammatory hepatocellular adenoma with atypical features. The imaging features of hepatocellular adenoma and focal nodular hyperplasia are similar, but if the tumor tends to grow, surgical treatment should be performed because of the possibility of malignant hepatocellular adenoma.

We report valuable imaging findings in a case of β-catenin-activated hepatocellular adenoma (β-HCA) with weak β-catenin activation. A 40 year-old female presented with a liver tumor in S8 that was incidentally detected on ultrasonography. The tumor showed marked enhancement and early venous drainage into the middle hepatic vein in the arterial phase of contrast-enhanced computed tomography (CT). The tumor revealed slight hypointensity in the hepatobiliary phase of gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging (EOB-MRI). Six months after detection, the tumor had increased in size and a biopsy indicated hepatocellular carcinoma. The tumor was resected and pathologically diagnosed as β-HCA with weak β-catenin activation such as exon 3 S45 mutation and exon 7/8 mutation. Marked enhancement in the arterial phase of CT and MRI is a characteristic finding of β-HCA with weak β-catenin activation. Furthermore, the degree of β-catenin activation might determine the signal intensity of β-HCA in the hepatobiliary phase of EOB-MRI.

Hepatocellular adenomas (HCA) in infants are exceedingly rare with only 5 cases reported to the best of our knowledge, all of them preceding the classification of HCA. Here we present an autopsy case of a 9-month-old girl with Burn-McKeown syndrome with an incidental liver nodule in the right lobe measuring 1.5 cm in greatest dimension. The lesion was composed of an unencapsulated proliferation of hepatocytes with multiple unaccompanied arteries without well-formed portal tracts, and an intact reticulin framework without thickened hepatic plates, findings consistent with an HCA. Glutamine synthetase (GS), lipid fatty acid-binding protein (LFABP), c-reactive protein (CRP), serum amyloid-a (SAA), beta-catenin and CD34 immunostains were performed. GS was diffusely and strongly positive in the lesion, CD34 showed heterogenous staining of sinusoids within the lesion without a well-formed rim from the background liver and beta-catenin was negative for nuclear staining. CRP and SAA were considered negative, and LFABP was retained. Molecular testing showed no CTNNB1 variants and found two tier 3 variants involving CHEK2 and PTEN genes. These findings are consistent with an unclassified HCA (U-HCA) per the 2019 WHO Classification of Tumors, representing the youngest patient reported. This raises the possibility that some HCAs are congenital or develop very early in life, remaining undiagnosed until later in life.

Hepatocellular adenoma (HCA) is a benign hepatocyte-derived epithelial tumor. HCA is associated with oral contraceptive use among Caucasian populations. We report a case of hepatocellular adenoma with a pedunculated protuberance and high protein induced by vitamin K absence or antagonist-II (PIVKA-II) levels, which made diagnosis challenging. The patient was a 22-year-old woman. In a medical check-up, a high γ-GTP level was detected and a 115-mm solid mass was found in her lower abdomen via abdominal ultrasonography. A blood test showed a high PIVKA-II level. Abdominal CT showed a tumor in the lower abdomen. Contrast-enhanced CT showed a blood vessel thought to be the left hepatic artery connecting to the mass, and a blood vessel thought to be the left hepatic vein returning from the mass to the inferior vena cava. In EOB-MRI, uneven enhancement was observed after contrast imaging, but washout in the equilibrium phase was unclear. Parenchymal hepatocyte phases showed a pale, non-uniform, high signal. These findings indicated that the tumor was derived from the left lobe of the liver and was suggestive of HCC. Surgical resection was then performed. A pathological examination led to a diagnosis of HCA, corresponding to unclassified HCA. The WHO classification of tumors of the digestive system based on an immunohistological examination includes HNF1α-inactivated HCA, β-catenin-activated HCA, inflammatory HCA, and unclassified HCA. In summary, our patient had a large HCA with pedunculated protrusion into the extrahepatic pelvic cavity. This case was challenging to diagnose because of abnormally high PIVKA-II levels, and it was resected laparoscopically.

BACKGROUND: Focal nodular hyperplasia (FNH) and hepatocellular adenoma (HCA) are well-known benign liver lesions. Surgical treatment is usually chosen for symptomatic patients, lesions more than 5 cm, and uncertainty of diagnosis.
METHODS: We described the case of a large liver composite tumor in an asymptomatic 34-year-old female under oral contraceptive for 17-years. The imaging work-out described two components in this liver tumor; measuring 6 cm × 6 cm and 14 cm × 12 cm × 6 cm. The multidisciplinary team suggested surgery for this young woman with an unclear HCA diagnosis. She underwent a laparoscopic left liver lobectomy, with an uneventful postoperative course. Final pathological examination confirmed FNH associated with a large HCA. This manuscript aimed to make a literature review of the current management in this particular situation of large simultaneous benign liver tumors.
CONCLUSIONS: The simultaneous presence of benign composite liver tumors is rare. This case highlights the management in a multidisciplinary team setting.

UNASSIGNED: Hepatocellular adenoma (HCA) is a rare liver tumor. We report a case of a radio-resistant liver tumor that was removed surgically and found to be HCA.
METHODS: A 37-year-old Japanese man was incidentally diagnosed with a liver tumor. He had no history of viral hepatitis nor metabolic disorders. MRI revealed a tumor enhancing in arterial phase, followed by washout in late phase, and hypointensity in hepatobiliary phase. A diagnosis of hepatocellular carcinoma (HCC) was made and surgery was advised. However, the patient chose proton beam radiotherapy. Although the tumor initially shrunk, it increased in size thereafter. Therefore, anterior sectionectomy was performed. Histology revealed proliferation of hepatocytes without cytologic atypia. On immunohistochemistry, CRP, SAA, GS, L-FABP, and nuclear expression of β-catenin were positive. A final diagnosis of mixed inflammatory and β-catenin activated HCA was made.
UNASSIGNED: HCA is associated with obesity. The present case was a slightly obese man without history of viral hepatitis. In such cases, HCA should be considered. In the present case, proton beam radiotherapy was performed for a diagnosis of HCC. However, the tumor was radio-resistant.
CONCLUSIONS: HCA shows an almost equal male to female ratio in the Asian population. Molecular classification is vital in the management of HCA. HCC and HCA are often difficult to differentiate; tumor biopsy is necessary for patients with atypical imaging findings and in younger patients without underlying liver disease. Since the effectiveness of radiation therapy on HCA has not been reported, surgery should be preferred.