Management of benign liver tumors.

Benign liver tumors in children are far less frequent than their malignant counterparts. Recently, there have been advances of diagnostic procedures and novel treatments with improved classification as a result. While malignant pediatric liver tumors have been comprehensively addressed by multicenter international tumor trials, benign tumors have more usually relied upon individualised workup and treatment. Due to the rarity and heterogeneity of these different entities, large studies are lacking. In this article the authors highlight the spectrum of benign liver tumors with special focus on specific clinical features, pathology, and treatment.

Measuring hepatic functional reserve using T1 mapping of Gd-EOB-DTPA enhanced 3T MR imaging: A preliminary study comparing with 99mTc GSA scintigraphy and signal intensity based parameters.

PURPOSE: To determine the utility of liver T1-mapping on gadolinium-ethoxybenzyl-diethylenetriamine-pentaacetic acid (Gd-EOB-DTPA) enhanced magnetic resonance (MR) imaging for the measurement of liver functional reserve compared with the signal intensity (SI) based parameters, technetium-99m-galactosyl serum albumin (99mTc-GSA) scintigraphy and indocyanine green (ICG) clearance. MATERIALS AND METHODS: This retrospective study included 111 patients (Child-Pugh-A 90; -B 21) performed with both Gd-EOB-DTPA enhanced liver MR imaging and 99mTc-GSA (76 patients with ICG). Receiver operating characteristic (ROC) curve analysis was performed to compare diagnostic performances of T1-relaxation-time parameters [pre-(T1pre) and post-contrast (T1hb) Gd-EOB-DTPA], SI based parameters [relative enhancement (RE), liver-to-muscle-ratio (LMR), liver-to-spleen-ratio (LSR)] and 99mTc-GSA scintigraphy blood clearance index (HH15)] for Child-Pugh classification. Pearson's correlation was used for comparisons among T1-relaxation-time parameters, SI-based parameters, HH15 and ICG. RESULTS: A significant difference was obtained for Child-Pugh classification with T1hb, ΔT1, all SI based parameters and HH15. T1hb had the highest AUC followed by RE, LMR, LSR, ΔT1, HH15 and T1pre. The correlation coefficients with HH15 were T1pre 0.22, T1hb 0.53, ΔT1 -0.38 of T1 relaxation parameters; RE -0.44, LMR -0.45, LSR -0.43 of SI-based parameters. T1hb was highest for correlation with HH15. The correlation coefficients with ICG were T1pre 0.29, T1hb 0.64, ΔT1 -0.42 of T1 relaxation parameters; RE -0.50, LMR -0.61, LSR -0.58 of SI-based parameters; 0.64 of HH15. Both T1hb and HH15 were highest for correlation with ICG. CONCLUSION: T1 relaxation time at post-contrast of Gd-EOB-DTPA (T1hb) was strongly correlated with ICG clearance and moderately correlated HH15 with 99mTc-GSA. T1hb has the potential to provide robust parameter of liver functional reserve.

Visualization of draining vein in focal nodular hyperplasia by superb microvascular imaging: report of two cases.

Focal nodular hyperplasia (FNH) is the second most common benign tumor of the liver, and diagnosis and follow-up without invasive procedures are now highly recommended. FNH shows a characteristic vascular structure called "spoke-wheel appearance". However, this appearance relies on the mode of the feeding artery, and the mode of the draining vein has not been sufficiently studied until recently. Superb microvascular imaging (SMI) is a new Doppler technology designed to improve visualization of the blood flow, especially slow flow signals from fine vessels, using a new algorithm. Thus, we can expect to see an improvement in visualization of fine vessels in FNH using SMI. Here, we present two cases in which SMI clearly demonstrated not only the typical spoke-wheel appearance but also draining to the hepatic veins. Our observation is believed to be noteworthy in the diagnosis of FNH. In conclusion, we have reported two cases of hepatic FNH in which SMI revealed clearly not only its feeding vessels and intratumoral vasculature but also its draining vessels communicating with the hepatic vein. SMI is considered to increase our diagnostic confidence by revealing fine vessels within and around the FNH lesion.

Focal nodular hyperplasia: our experience of 53 Japanese cases.

PURPOSE: Contrast-enhanced ultrasound (CEUS) is a very sensitive diagnostic tool in characterizing liver tumors. It is especially useful in the diagnosis of focal nodular hyperplasia (FNH) of the liver. According to the previous reports, FNH is common in young women, and it is usually diagnosed by MRI. The majority of the previous reports come from European countries, and a very few studies of large series designed to describe the clinical features in Japanese patients have been reported. The aim of this study was to (a) describe the clinical features in 53 patients (59 lesions) diagnosed with CEUS and (b) compare the data with those from the previous reports. METHODS: The medical data from 53 patients diagnosed on the basis of typical CEUS findings at our institution and affiliated hospitals were reviewed, and their clinical data were analyzed. RESULTS: (1) The medical data from 53 cases showed a slight male predilection, with 30 male cases (57 %) and 23 female cases (43 %), although the occurrence in both sexes was equal. FNH cases were distributed throughout all generations in both sexes, mostly concentrated in the age of 30-60 years old, and metabolic cases were more common in men than in women (4 vs 0). (2) The lesions were small (mean: 23 mm) and distributed throughout the whole liver. (3) Lesion size was not influenced by age in either sex. (4) A rapid draining to the hepatic vein was recognized in five out of 59 lesions (8 %). CONCLUSIONS: Our data indicate that FNH occurs slightly more frequently in men than in women in Japan. It occurs also at any age in both sexes, but the mean lesion size was smaller in our series than in the previous reports. Metabolic disease was seen only in male FNH patients. A direct communication between the FNH lesion and the hepatic vein is diagnostically worth noting.

[Focal nodular liver hyperplasia].

It was evaluated the frequency of focal nodular liver hyperplasia and treatment resultsin 1425 patients for the last 17 years in 2 time period. The own experience and literature data were used. First this disease was considered as very rare liver tumor because of underdeveloped diagnostics. Tumor detection was the indication for laparotomy. In some patients removal of the tumor was not performed if intraoperativebiopsy has shown tumor benign. The indication for dynamic observation and surgical treatment were identified with increasing number of patients, diagnostics improvement, and analysis of immediate and long-term results. The operation provides persistentcure.

Focal nodular hyperplasia and hepatic adenoma: current diagnosis and management.

Benign liver tumors are common lesions that can be classified into cystic and solid lesions. Cystic lesions are the most frequent; however, they rarely represent a diagnostic or therapeutic challenge. In contrast, solid lesions are more difficult to characterize and management remains controversial. The wide availability and use of advanced imaging modalities, including ultrasonography, computed tomography, and magnetic resonance imaging have led to increased identification of incidental liver masses. Although some of these incidentally discovered masses are malignant, most are benign and must be included in the differential diagnosis. In this article we review FNH and HA. Its etiology, biological behavior, diagnosis, and treatment will be highlighted.

Development of multiple focal nodular hyperplasia lesions after portocaval shunting. A case report.

It has been postulated that altered hepatic blood flow, particularly reduced portal flow, is responsible for the induction of hyperplasia of liver cells and nodule formation. This report describes the case of a 31-year old female patient developing multiple focal nodular hyperplasia (FNH) lesions two years after portocaval shunting and extended right hemihepatectomy due to the suspicion of a malignant liver tumor. Portocaval shunting became necessary due to iatrogenic thrombosis of the entire portal vein after preoperative embolization of the right portal vein. This observation provides for the first time direct evidence for the pathogenesis of FNH in humans.

Hepatic hemodynamics in 24 patients with nodular regenerative hyperplasia and symptomatic portal hypertension.

BACKGROUND AND AIM: To evaluate hepatic hemodynamics in patients with nodular regenerative hyperplasia of the liver (NRH) with portal hypertension (PHT). METHODS: We retrospectively reviewed the charts of 24 patients referred for PHT related to biopsy-proven NRH. Hemodynamic measurements included wedged hepatic vein (WHVP) and inferior vena cava (IVCP), and, in 12 patients, portal vein pressure (PVP). Hepatic vein pressure gradient (HVPG: WHVP-IVCP) and portal vein pressure gradient (PVPG: PVP-IVCP) were calculated. RESULTS: Nodular regenerative hyperplasia was associated in 24 patients with various diseases (oxaliplatin chemotherapy, treatment with purine antagonists, post liver transplantation, hematologic and rheumatologic conditions and HIV infection). Liver function parameters were either completely normal or slightly impaired. Patients were referred for gastroesophageal varices (n = 18), and/or ascites (n = 11), and/or splenomegaly (n = 20). In patients with varices or ascites, HVPG was lower than 10 mmHg (a cut-off point for the presence of varices and/or ascites) in 15/21, suggesting a pre-sinusoidal component to their PHT confirmed by a PVP higher than 12 mmHg in 12/12 patients. The mean difference between HVPG and PVPG was 8.7 mmHg in these patients. Ten patients were treated by transjugular intrahepatic portosystemic shunt. None of them re-bled, and one presented transient hepatic encephalopathy. CONCLUSIONS: Presinusoidal PHT associated with NRH is probably related to compression of portal venules by the regenerative nodules. In patients with HTP and a HVPG < 10 mmHg, the diagnosis of NRH must be suspected and PVP measured, which is important in the management of these patients.

Hipertensión arterial maligna e hiperaldosteronismo hiperreninémico: reporte de un caso / Malignant arterial hypertension and hyperreninemic hyperaldosteronism: a case report

Se presenta el caso de una mujer de 28 años de edad, hospitalizada por hipertensión arterial severa, edema pulmonar e hipokalemia. se encontró hiperaldosteronismo hiperreninémico e imagen de una lesión nodular en la glándula suprarrenal izquierda, la cual fue extirpada mediante cirugía laparoscópica. El estudio histológico demostró hiperplasia macronodular cortical y en el postoperatorio, hubo retorno progresivo de la hipertensión e hipokalemia

The beta-catenin pathway is activated in focal nodular hyperplasia but not in cirrhotic FNH-like nodules.

BACKGROUND/AIMS: Focal nodular hyperplasias (FNHs) are benign liver lesions considered to be a hyperplastic response to increased blood flow in normal liver. In contrast, FNH-like lesions/nodules occur in cirrhotic liver but share similar histopathological features. We conducted a transcriptome analysis to identify biological pathways deregulated in FNH. METHODS: Gene expression profiles obtained in FNH and normal livers were compared. Differentially-expressed genes were validated using quantitative-RT-PCR in 70 benign liver tumors including FNH-like lesions. RESULTS: Among the deregulated genes in FNHs, 19 displayed physiological restricted distribution in the normal liver. All six perivenous genes were up-regulated in FNH, whereas 13 periportal genes were down-regulated. Almost all these genes are known to be regulated by beta-catenin. Glutamine synthetase was markedly overexpressed in anastomosed areas usually centered on visible veins. Moreover, activated hypophosphorylated beta-catenin protein accumulated in FNH in the absence of activating mutations. These results suggest the zonated activation of the beta-catenin pathway in FNH, whereas the other benign hepatocellular tumors, including FNH-like lesions, demonstrated an entirely different pattern of beta-catenin expression. CONCLUSIONS: In FNH, increased activation of the beta-catenin pathway was found restricted to enlarged perivenous areas. FNH-like nodules may have a different pathogenetic origin.

The involvement of the liver in systemic diseases.

The liver has a double blood supply and plays a central role in the metabolism of proteins, carbohydrates, and many medications. In addition, it has a role in the induction of immune tolerance and may also be a target for immune-mediated damage. For these reasons, the liver may be involved in many systemic diseases. In this review, we discuss the involvement of the liver in granulomatous, rheumatologic, malignant, and circulatory diseases. An understanding of the wide spectrum of liver involvement in systemic diseases will aid in both diagnosis and treatment of patients with a wide range of medical conditions.

Hiperplasia nodular focal do fígado: apresentação de um caso e revisão da literatura / Focal nodular hyperplasia of the liver: a case report and review of the literature

Neste trabalho apresentamos um caso de hiperplasia nodular focal que foi diagnosticado aos seis anos de idade e que está sendo acompanhado até o momento presente. Para o diagnóstico foram imprescindíveis as técnicas de imagem, tendo importância de realce a cintilografia hepatoesplênica e a tomografia computadorizada. Apresentamos, também, revisão da literatura sobre o assunto.

In this case report we discuss a focal nodular hyperplasia diagnosed in a female, six-year old patient, as well as her follow-up from the diagnosis to the present time. Imaging techniques, particularly hepatosplenic scintigraphy and computed tomography, are essential for the diagnosis. Also, a literature review is presented.

Focal nodular hyperplasia and hepatic adenoma: a review.

Focal Nodular Hyperplasia (FNH) and Hepatic Adenoma (HA) are a benign tumors of the liver. The association with the use of oral contraception in women in middle age has been mentioned. This benign liver tumors are relatively rare lesions and are usually unrelated to subjective symptoms. They are increasingly being diagnosed as a result of the widespread use of ultrasound, computed tomography and magnetic resonance in the evaluation of patients with non-specific abdominal symptoms.

Nodular regenerative hyperplasia of the liver: a case report.

Nodular regenerative hyperplasia of the liver is characterized by hepatocellular nodules distributed throughout the liver in the absence of fibrous septa between the nodules. In the literature, most reports have been single cases so that the prevalence and clinical significance of the disease is not exactly known. The critical lesion is obliterative portal venopathy which is obstruction to terminal radicals of hepatic arterioles and portal venules, possibly secondary to endothelial cell damage. Resultant hepatic ischemia may be responsible for induction of the nodular regenerative change. Portal hypertension which is secondary to nodular regenerative hyperplasia and is often complicated by bleeding esophageal varices. We report a rare case of nodular regarative hyperplasia of the liver, with onset of symptom in childhood.

A quantitative gene expression study suggests a role for angiopoietins in focal nodular hyperplasia.

BACKGROUND AND AIMS: Although the pathogenesis of focal nodular hyperplasia (FNH) of the liver remains unclear, a vascular mechanism has been suspected. To gain insight into the pathogenesis of FNH, we performed a large-scale quantitative study of gene expression in FNH. METHODS: Quantitative expression level of 209 selected genes was assessed using real-time reverse-transcription polymerase chain reaction in 14 cases of FNH and compared with their expression level in 13 cases of liver cirrhosis, 4 adenomas, and 15 hepatocellular carcinomas. RESULTS: Among the 7 genes, the expression of which was significantly up-regulated or down-regulated in FNH, the most informative markers for the diagnosis of FNH as assessed using the receiving operative curve and area under the curve (AUC) were angiopoietin-1 (Ang-1; AUC, 0.82) and angiopoietin-2 (Ang-2; AUC, 0.80). These 2 genes are involved in the regulation of vasculogenesis. In FNH, Ang-1 was significantly up-regulated, Ang-2 was down-regulated, and the Ang-1/Ang-2 ratio was highly and specifically increased in FNH compared with normal liver or other groups of lesions (FNH, 15.2-fold increase; HCC, 2.78; adenoma, 2.28; cirrhosis, 1.92; P < 0.01 for FNH vs. all groups, analysis of variance). Tie-2 messenger RNA, the receptor of Ang-1 and Ang-2, was detected at the same level in FNH as in normal liver. Ang-1 protein was detected on Western blot of FNH and expressed by endothelial cells of dystrophic vessels and sinusoids as shown by immunohistochemistry. CONCLUSIONS: A specific increase of Ang-1/Ang-2 ratio in FNH, in the presence of the functional Tie-2 receptor, might be involved in the formation of hyperplastic and dystrophic vessels of FNH.