Magnetic Resonance Imaging in Focal Liver Lesions with Diffusion Weighted Imaging (DWI) and Pathological Correlation

Background: Magnetic resonance signal intensity of focal liver lesions is affected by numerous pathologic factors. Lesion histologic features, such as cellularity, vascularity, stromal component, and intratumoral necrosis or hemorrhage, strongly affect T1 and T2 relaxation times. Aim and objectives: To assess the lesion characterization potential of MRI by evaluating unenhanced and dynamic gadolinium enhanced sequences, Histopathological correlation of the lesions to explain the major MRI findings and Assessment of the lesions by diffusion weighted imaging and investigating the role of b value in differentiating malignant and benign lesions Materials and methods: The study was done on 42 patients where contrast was given in 38 members. Focal liver lesions were analyzed based on clinical findings, laboratory investigations and MR imaging. Sonographically detected focal lesions were included in the study. Tissue diagnosis (FNAC/ Biopsy), surgery were done in feasible cases. In other cases, where surgery/ tissue diagnosis is not possible, follow up was done (range 3 months - 12 months, average 7.2 months). Results: MRI features of forty two patients with focal liver lesions were studied and morphological features and signal intensities of the masses were described. Contrast study was done in 39 cases. Of the 42 cases, 24 lesions were benign and 18 lesions were malignant. The mean age group ranged from 18 to 74 years with majority between 40 to 60 years. 61% of the lesions were located in right lobe of the liver. Contrast enhancement was done in 39 cases. Contrast enhancement was able to better delineate the cases. Specific pattern of contrast enhancement was typical of certain lesions as Karuna V, R Vikash Babu. Magnetic Resonance Imaging in Focal Liver Lesions with Diffusion Weighted Imaging (DWI) and Pathological Correlation. IAIM, 2019; 6(11): 81-93. Page 82 homogenous early arterial phase enhancement for hepatocellular carcinoma and ring enhancement in arterial phase for metastases. Delayed enhancement was specific for cholangiocarcinoma. Hemangiomas showed peripheral puddling and delayed central enhancement. Diffusion weighted imaging can be used as an additional tool in differentiating benign and malignant focal liver lesions. ADC value using a cut-off of 1.43 X 10 – 3 sec/ mm2 was a useful adjunct for determining benign cystic lesions and hemangiomas from malignant lesions. Conclusion: MRI was able to predict diagnosis in 38 of the 42 tumors. It could suggest the nature of all lesions in benign cysts, hemangiomas, focal nodular hyperplasia and metastases. But it was not possible to achieve a specific diagnosis in two early abscesses, one multifocal hepatocellular carcinoma and one case of regenerative nodules. This accounted for a detection rate of 90%. Thus MR imaging is a powerful tool for the evaluation of focal liver lesions. Pre contrast T1 weighted gradient echo images, T2 weighted images, in phase and out phase imaging, EPI – DWI and gadolinium enhanced T1 weighted images provide accurate characterization of the lesions.

Case report of hepatic adenoma with hemorrhage in glycogen storage disease type Ⅰa / 基础医学与临床

Objective To analyze and summarize the clinical characteristics of patients with spontaneous hemor -rhage of hepatic adenoma in glycogen storage disease type Ⅰa.Methods Reporting 1 case in our hospital and making a summary about general situation , category, etiology, diagnosis and treatment of the hemorrhage of hepatic adenoma with glycogen storage disease type Ⅰa through checking literatures .Results The patient was a 27 year old male who had been diagnosed as glycogen storage disease for 14 years, as well as was first found hepatic adeno-ma at the age of 17 .He once was diagnosed as intra-adenoma bleeding with persistent abdominal pain and dizziness and was underwent selective hepatic artery embolization at the age of 22.Hepatic adenoma in glycogen storage dis-ease typeⅠa generally appeared at the age of puberty .One common complication of this disease was hemorrhage of hepatic adenoma , which can be found by ultrasonography and CT .Clinical management includs observation , selec-tive hepatic artery embolization , radiofrequency ablation , surgical resection and liver transplantation .Conclusions Glycogen storage disease type Ⅰa is an autosomal recessive genetic disease with hepatic adenoma as a common complication of GSD Ⅰa, serious liver adenoma's hemorrhage can be life threatening , the radiological examination can be helpful to detect hepatic adenoma .Then appropriate intervention can improve the life quality and prognosis .

A case of focal nodular hyperplasia with growth progression during pregnancy

Focal nodular hyperplasia (FNH) is the second most common benign solid tumor of the liver and is usually found in young females. In FNH, spontaneous bleeding or rupture rarely occurs and malignant transformation is unlikely. The etiology of FNH is unclear, but because of female predominance and young age at onset, it seems that female hormone has an important role for the development of FNH. Although the development and the complications of hepatocellular adenomas have been related to the use of oral contraceptives and pregnancy, the influence of oral contraceptives and pregnancy on the growth and complications of FNH is controversial. Most FNH are stable in size and rarely complicated during pregnancy. We describe here a case of FNH with growth progression during pregnancy in a 27-year-old female. Her course of pregnancy and delivery was uneventful. Two months after delivery, the size of FNH was decreased.

Adenoma hepático / Hepatic adenoma

INTRODUÇÃO: Os tumores hepáticos benignos, devido à relativa facilidade na identificação através de exames de imagem, tiveram incidência crescente na população nos últimos anos, tornando-se quadro frequente na rotina clínica e muitas vezes um desafio para clínicos e cirurgiões. Logo, o médico passou a enfrentar dilemas relacionados ao diagnóstico e conduta nestes pacientes assintomáticos e portadores de nódulos hepáticos. OBJETIVO: Atualizar o conhecimento dos adenomas hepáticos frente à evolução ocorrida com seu conhecimento nos últimos anos. MÉTODO: Foi efetuada revisão da literatura em consulta na Medline/Pubmed, Scielo, Embase e Lilacs com cruzamento dos seguintes descritores: adenoma hepático, cirurgia, tratamento clínico, diagnóstico, fisiopatologia e biologia molecular. CONCLUSÃO: O diagnóstico incidental de lesões assintomáticas constitui grande dilema na prática clínica, pois traz intensa angústia para o paciente e seus familiares, e muitas vezes tornam-se um desafio para o clínico ou cirurgião. É lesão de particular interesse, pois pode apresentar evolução tanto benigna como complicações potencialmente letais. O adenoma hepático deixou recentemente de ser lesão de ressecção obrigatória; atualmente, adota-se conduta mais individualizada, visando menor morbimortalidade. À luz dos novos avanços da biologia molecular, cabe ao médico que o diagnostica identificar aqueles com potencial evolução desfavorável, para que nesses seja empregada conduta mais agressiva.

BACKGROUND: Benign liver tumors, due to its relative easeness its imaging identification, have their incidence increasing in population in recent years, becoming frequent in the clinical picture and often a challenge for clinicians and surgeons. Doctors began to face dilemmas related to diagnosis in asymptomatic patients with liver nodules. AIM: Update the knowledge of hepatic adenomas due to the crescent diagnosis seen in the recent years. METHODS: Was performed a literature review consulting Medline/PubMed, SciELO, Embase, Lilacs database with the following descriptors: hepatic adenoma, surgery, medical treatment, diagnosis, pathophysiology and molecular biology. CONCLUSION: The diagnosis of incidental asymptomatic lesions is a major dilemma in clinical practice because it brings intense distress for patients and their families, and often become a challenge for the physician or surgeon. Injury is of particular interest because it can provide both benign evolution or potentially lethal complications. Recently, its resection is no more mandatory; currently, more individualized treatment are required, aiming less morbidity. In light of new advances in molecular biology, the physician who diagnoses the lesion must identify the potential unfavorable evolution, and recognize cases who need more aggressive medical management.

Characteristic sonographic findings of liver and kidney in patients with glycogen storage disease type I: An analysis of 2 cases / 第二军医大学学报

Objective To analyze the characteristic sonographic findings of the liver and kidney in patients with glycogen storage disease type I (GSDI). Methods The clinical data of 2 children, who were pathologically diagnosed as having GSD I in our hospital, were retrospectively analyzed, and the sonographic findings of the liver and kidney were given special attention. Results The two patients were both females, one aged 11 years old and the other aged 19. Both of them had diffused hepatomegaly with meticulous and enhanced internal echoes. One had multiple lesions in the liver, which were proven to be hepatic adenomas, with diffuse enhanced cortical echogenicity in both kidneys, but with no renal calculus. Both patients had a small-sized thyroid and uterus, with short stature and liver dysfunction as clinical presentations. Conclusion A better understanding of the ultrasonic findings of liver and kidneys in GSD I patients can benefit the timely discovery and diagnosis of this systematic metabolic disease.

A case of multiple hepatic adenomas and gout with glycogen storage disease type Ia / 대한내과학회지

Glycogen storage disease (GSD) type Ia is a rare inherited metabolic disease characterized by glucose-6-phosphatase (G6Pase) deficiency, which results in many metabolic problems, such as fasting hypoglycemia, lactic acidosis, hyperuricemia, and hyperlipidemia. The metabolic derangements may result in long-term complications, including growth retardation, gout, hepatic adenomas, and renal disease. A 26-year-old male was admitted with general weakness, multiple subcutaneous mass-like lesions, and hepatomegaly. He was diagnosed as GSD type Ia through analysis of the G6Pase gene. This disease is found mainly in childhood, but we diagnosed a case of GSD type Ia during a work-up of arthralgia and hepatomegaly in an adult patient. We report this case with a review of the literature

Long-term Outcome of Glycogen Storage Disease Type 1; Analysis of Risk Factors for Hepatic Adenoma / 대한소아소화기영양학회지

PURPOSE: The aim of the study was to evaluate the long-term outcome of glycogen storage disease (GSD) type 1 with particular reference to hepatic adenoma and hepatocellular carcinoma, and to analyze risk factors affecting the development of hepatic adenoma in GSD type 1. METHODS: Forty-three GSD type 1 patients (31 males and 12 females, mean age 13.9+/-6.4 years) were analyzed retrospectively. Hepatic adenoma was detected on abdominal USG and diagnosed on histologic examination. Clinical profiles were compared between patients with hepatic adenoma (n=16) and age-matched controls without hepatic adenoma (n=16). RESULTS: 1) Of 43 GSD type 1 patients, 16 (37.2%) had hepatic adeoma. Hepatic adenoma was detected at the age of mean 14.2+/-4.1 years (range: 7.9~25.7 years). Fourteen (87.5%) adenomas were multiple at detection. 2) Comparison of the clinical profiles between adenoma group and non-adenoma group revealed that age at first introduction of uncooked cornstarch treatment was significantly late in adenoma group compared with non-adenoma group (9.1+/-5.2 years vs. 3.0+/-1.8 years, p=0.003). Portocaval shunt surgery was performed in 11 (68.8%) patients in adenoma group and 3 (18.8%) in non-adenoma group (p=0.004). Hepatic adenoma developed mean 5.8+/-4.2 years after shunt operation. 3) One patient was diagnosed as hepatocellular carcinoma at the age of 25.7 years. CONCLUSION: Hepatic adenoma is an important late complication of GSD type 1 with the risk of malignant transformation. Early introduction of cornstarch therapy with strict metabolic control is needed to prevent the development of hepatic adenoma in GSD type 1.

Clinical Review of Hepatic Adenoma reported in Korea / 대한내과학회지

OBJECTIVES: Hepatic adenoma is a rare benign tumor, occuring in women of child-bearing age and it is known that hepatic adenoma is associated with history of oral contraceptive use in about 90% of cases, rarely with type I glycogen storage disease or anabolic steroids. There have been five cases of hepatic adenoma reported in Korea and three cases have been confirmed in our hospital. We examined the clinical findings of these eight cases and compare them with those of hepatic adenoma reported in western countries. METHODS: We reviewed five cases published in medical literature of Korea and three cases confirmed in our hospital. The clinical, laboratory, and radiological features of the eight cases were examined and all were pathologically proven cases as hepatic adenoma. RESULTS: Six cases were male and two cases were female. Mean age of them were 37+/-6 years old which ranged from 24 to 45 years old. Two cases of female did not have history of ingestion of oral contraceptives and six cases of male did not have history of having anabolic steroids. The associated diseases were chronic hepatitis B in two cases, glycogen storage disease type I in one case, and ovarian mucinous cystadenoma in one case. The presenting symptoms were upper abdominal discomfort in five cases, chronic fatigue in one case and two cases had no symptom. Hepatic adenomas were single mass in seven cases and only one case suspected of having glycogen storage disease showed six nodules. The mean diameter of the eight hepatic adenoma was 7.7+/-3.7cm which range from 2.0cm to 15cm. Seven of the eight cases were larger than 5cm in diameter. but there was no case of intraperitoneal bleeding. In laboratory studies, AST/ALT was elevated(81/84IU/L, normal range less than 40IU/L) in one case which was suspected of glycogen storage disease. Alkaline phosphatase were elevated in four cases and elevated alpha-FP was not found in any case. Hepatic adenoma was shown as hyperechoic mass with indistinct margin by ultrasonography and hypodense mass with contrast enhancement by computed tomography. Hepatic adenoma showed isodense or low signal intensity in T1WI, and low or high signal intensity in T2WI. Hepatic angiography showed hypervascular mass in six cases but avascular mass in two cases. CONCLUSION: The reported cases of hepatic adenoma were predominant in men, associated with chronic hepatitis B in two cases, and females with hepatic adenoma had no history of having oral contraceptive. Despite the large size of hepatic adenoma, there was no case accompanied by peritoneal bleeding. These findings contrasts with those of hepatic adenoma reported in western countries. Therefore the pathogenesis and clinical course of hepatic adenoma in Korea may be different with those of hepatic adenoma occurring in western countries and further studies are needed.