Liver In Infancy Research Articles

A rare case of non-treated fetal–type hepatoblastoma for five years stayed without metastases in a young boy

Hepatoblastoma is the most common malignant neoplasm of the liver in infancy. In this paper, we present a rare case of non-treated hepatoblastoma in an 18-month-old baby male who presented with an asymptomatic abdominal mass associated with elevated Alpha-fetoprotein. Contrast-enhanced CT revealed a solid mass located in the left lobe of the liver, measuring about 6 × 6 cm, and not enhancing the contrast. US-guided Tru-cut biopsy was performed which showed pure epithelial fetal-type hepatoblastoma. The parents refused neoadjuvant chemotherapy and the connection was disconnected with them because of the ongoing crisis in Syria. After five years, the patient returned with the same clinical picture. CT scan was repeated which revealed the same tumor without metastases. The decision was made to do a left hepatectomy after another confirmation with a surgical biopsy. Histopathological examination showed a multifocal pure epithelial-fetal well-differentiated type of hepatoblastoma with a favorable grade. Moreover, surgical margins and liver capsule appeared free, and the tumor was considered in Stage 1 accordingly. To this date, the boy was followed up with serial US and Alpha-fetoprotein (AFP)levels without signs of recurrence. Hepatoblastoma with pure fetal-type histology can be treated with complete surgical excision without neoadjuvant or adjuvant chemotherapy if the tumor was resected completely because this type of neoplasm is slow growing and has a good prognosis.

Infantile Hepatic Hemangioma: Avoiding Unnecessary Invasive Procedures.

Infantile hepatic hemangioma, the most common vascular tumor of the liver in infancy, can occur with acute postnatal liver and congestive heart failure. Nevertheless, its course is often benign, and many children can be diagnosed and treated without surgical intervention. The distinction from malignant diseases is not always easy and it not clear whether invasive procedures for diagnosis and therapy should be performed. Here we report our experiences in our Center for Pediatric Liver Disease and postulate that large studies are needed to avoid unnecessary invasive procedures for these patients in the future.

Infantile hepatic hemangioma: current state of the art, controversies, and perspectives.

Infantile hepatic hemangioma (IHH) is a common vascular tumor, distinctive for its perinatal presentation, rapid growth during the first year of life, and subsequent involution. Although they generally follow a benign course, some tumors have been reported to undergo malignant transformation. The diagnosis of IHH is based on patient's medical history, physical examination, and imaging. Moreover, the management of this vascular tumor is based on clinical presentation and includes observational, medical, surgical, and radiological interventional treatment options. The present review presents the currently available data in the literature on the diverse aspects of the terminology, epidemiology, clinical presentation, pathogenesis, diagnosis, indications for surgery, malignant potential, and long-term outcomes of these tumors.Conclusion: No formal guidelines have yet been established for the treatment of these hepatic lesions, and the therapeutic strategies implemented vary widely from simple observation to medical, radiological, and surgical interventions in the prism of multidisciplinary teams.What is Known:• Infantile hepatic hemangioma is the most common benign tumor of the liver in infancy, but despite its benign nature, it can present with life-threatening complications.• The treatment strategies range from simple observation to a series of medical, surgical, and radiological interventions.What is New:• This review gives an overview of the developments and current status about the management of IHH.• The aim of this study is to clear up the confusion and controversy that exists about terminology, diagnosis, and treatment of IHH.

Infantile hemangioendothelioma of the liver in a Nigerian newborn: A case report

Hepatic hemangioendothelioma is the most common benign vascular tumor of the liver in infancy and it is the most likely of all hepatic tumors to present with symptoms early in life, presenting in the first 6 months of life. It may present with life-threatening complications such as congestive cardiac failure and thrombocytopenia. As a way of creating awareness to the existence of this rare condition in our environment, we report a case of symptomatic hemangioendothelioma in a newborn and challenges that can be encountered during management in a resource poor-setting. Baby OA is a 22-day-old female neonate who presented with an abdominal mass noted at birth. Diagnosis was made based on clinical findings and results of abdominal ultrasound scan and computerized tomography scan which were in keeping with a hepatic hemangioendothelioma. She was treated with oral prednisolone, propranolol, and aggressive supportive treatment with blood and platelet concentrate transfusions. Her management was hampered by challenges in obtaining a histologic diagnosis, financial constraints, and unavailability of wider therapeutic options. She has been discharged and is being followed-up at the out-patient clinics. Hemangioendothelioma should be considered in the differential diagnosis of infants presenting with an abdominal mass in the newborn period as it could be associated with significant morbidity and mortality requiring aggressive treatment and meticulous supportive care.

English

Infantile Hemangioendothelioma of the liver is an uncommon tumor. Two subtypes have been described. Type1 has recognizable but irregularly dilated vascular spaces in a fibromyxomatous stroma. Type 2 is described as having a more aggressive histology. It is marked by poorly formed and anatomising channels with papillae lined by atypical endothelial cells and is potentially malignant. Clinical presentation is variable, hepatomegaly or abdominal mass, some hematological abnormalities may be seen. In laboratory investigation, or lesion may be asymptomatic and discovered incidentally. We report a case of 45 day old female child who presented with abdominal distension. CT abdomen showed hypoattenuating mass in the left lobe of the liver along with specks of calcification.. The mass was excised and had large areas of gritty calcification simulating a teratoma. however histopathological diagnosis of infantile hemangioendothelioma type I of liver was made. Hemangioendothelioma of the liver has been reported as most common benign symptomatic vascular tumor of the liver in infancy.

Tratamiento exitoso con prednisona en un recién nacido crítico con hemangioendotelioma hepático infantil multifocal sintomático

Infantile hepatic hemangioendothelioma is the most common vascular tumor of the liver in infancy. The diagnosis can be enunciated without histological defnition, if the clinical, analytical and images fndings are very suggestive; particularly in children less than six months old, because the biopsy is not exempt of complications. We report a female patient, four days old and 3500 g, who developed a hypovolemic shock as a complication of the biopsy from a multifocal infantile hepatic hemangioendothelioma. She developed clinical manifestations of cardiac failure and thrombocytopenia. Because of the critical clinical status we choose the medical treatment with prednisone which was administered for fve month and complete remission of the tumor was obtained. We highlight the Doppler ultrasonography as a safe and no invasive diagnosis method. Management of this disease is commented as well.

Neonates with severe infantile hepatic hemangioendothelioma: Limitations of liver transplantation

IHHE as the most common vascular tumor of the liver in infancy can present with acute postnatal liver and congestive heart failure. LTx may be a lifesaving option, but can be complicated by extrahepatic involvement and bleeding complications, especially in neonates. Here we discuss the benefit of LTx in cases of acute postnatal deterioration and massive extent of the hepatic tumor. Three infants with IHHE were transplanted at our institution between 2005 and 2007. Two were neonates with acute postnatal decompensation and progressive liver and heart failure within days. Treatment with steroids and chemotherapy was ineffective; resection surgery and interventional treatment were not considered appropriate. LTx was performed at the age of 7 and 24 days, respectively. An additional infant with a bilobar tumor that evolved more slowly was transplanted on day-of-life 56. Patients 1 and 2 had to be resuscitated during the LTx procedure because of massive bleeding and both died during the procedure. Patient 3 had a complicated post-operative course but is doing well one-yr post-LTx. Neonates with extended hepatic and extrahepatic involvement of IHHE should be evaluated carefully prior to LTx. Whenever possible, alternative interventional treatment options should be considered.

Hepatic Haemangioendothelioma: A Vertical Association with Biliary Atresia?

Haemangioendotheliomas (HAE) are the commonest vascular tumours of the liver in infancy and are characterised by solitary or multifocal lesions often arising in conjunction with cutaneous haemangiomas. The authors report on a 4-month-old infant with massive hepatomegaly and hypothyroidism due to multifocal HAE whose mother had been born with biliary atresia. As the incidence of biliary atresia in the UK is about 1 in 16 000 live births, the association reported here seems more than co-incidental. This report reviews the literature and discusses potential aetiologies to explain this rare association.

Atypical imaging findings of infantile hemangioendothelioma: a case report

Infantile hemangioendothelioma is the third most common hepatic tumor in children, and the most common benign vascular tumor of the liver in infancy. On computed tomography (CT) and magnetic resonance imaging (MRI), typical enhancement patterns similar to those seen in adult hemangiomas may help establish the diagnosis. However, atypical enhancement patterns may also occur. In this paper, we report a rare form of solitary infantile hemangioendothelioma that showed a rim-like calcification and rim-like enhancement on CT and MRI.

Focal nodular hyperplasia of the liver in infancy: a case report

Focal nodular hyperplasia of the liver is a benign disorder usually affecting young women. Its treatment in children is traditionally conservative. Because of its rarity in childhood, its differential diagnosis with other hepatic tumors in children and infants is challenging. We present the case of a 7-month-old infant with large focal nodular hyperplasia affecting the whole left liver lobe. Because of progressive enlargement of the lesion and compression of the portal vein, the baby underwent successful surgical treatment. Different therapeutic options and relative indications are discussed.

Secretion and expression of epithelial markers supports the biliary origin of solitary nonparasitic cyst of the liver in infancy

Solitary nonparasitic cyst of the liver (SNPCL) is rare in children. Although there are several hypotheses regarding the pathogenesis, the true origin of SNPCL remains unknown. The authors present an infant with a huge SNPCL in whom the epithelial markers, CA19-9, DU-PAN-2, and SPan-1, were elevated in the serum and cystic fluid. The presence of CA19-9 and DU-PAN-2 was shown by immunohistochemistry in the cystic epithelia. These indicate that the classical idea of biliary origin of SNPCL is supported.

Congenital Solitary Nonparasitic Cyst of the Liver in Infancy and Childhood

Congenital Solitary Nonparasitic Cyst of the Liver in Infancy and Childhood

Giant hemangiomatosis of the liver in infancy

Giant hemangiomatosis of the liver in infancy

Diagnostic and Therapeutic Considerations of malignant tumors of the liver in infancy and childhood : with special reference to initial laboratory findings and chemotherapy

Diagnostic and Therapeutic Considerations of malignant tumors of the liver in infancy and childhood : with special reference to initial laboratory findings and chemotherapy

Normal size of liver in infancy and childhood. X-ray study.

The longest vertical distance between the upper and lower borders of the liver measured radiologically is suggested for estimating the size of the liver. This parameter, called vertical axis of the liver, was measured in 350 healthy infants and children, and the normal range at different age groups was defined. There was no correlation between palpability of the lower border and vertical axis, which shows that both parameters of the size of the liver depend on the position of the organ, apart from the size. Estimation of the vertical axis is suggested as a complementary measurement for cases where the palpability of the lower border of the liver gives values in the upper normal limits but where there are other reasons to suspect that the liver is enlarged.

The normal borders of the liver in infancy and childhood. Clinical and x-ray study.

In 365 healthy infants and children the normal range of the liver borders was defined by clinical and <i>x</i>-ray examination. It was found that in supine position and at the end of expiration the liver edge projects considerably below the costal margin in newborns and infants, and as the age increases the liver edge approaches the costal margin. In a considerable number of infants up to 6 months, the liver edge projects 3·0 to 3·5 cm. In children 10 to 16 years, the liver edge usually projects no more than 1 cm., though in occasional cases as much as 2 cm. below the costal margin. The clinical and <i>x</i>-ray examinations were in agreement in 95% of the cases.

Congenital cysts of the liver in infancy and childhood : P. W. Johnston. Amer. J. Surg. 116:184–191 (August) 1968

Congenital cysts of the liver in infancy and childhood : P. W. Johnston. Amer. J. Surg. 116:184–191 (August) 1968

Congenital cysts of the liver in infancy and childhood

A review of congenital cysts of the liver is made, including cases from the Childrens Hospital of Los Angeles, and those cases of cystic mesenchymal hamartoma and non-parasitic cysts in infancy and childhood which have been reported in the medical literature. The gross surgical findings and the operative procedures are emphasized, so that the surgeon encountering these lesions will be better able to decide on the nature of the lesion and on a safe and indicated operative procedure.

Renal lesions associated with cirrhosis of the liver in infancy and childhood.

IT IS well documented that alterations occur in the glomeruli of adults with hepatic disease. Salomon et al,1in a superb study of renal biopsies by light, electron, and fluorescence microscopy, demonstrated glomerular lesions regardless of the duration or nature of liver damage. Similar changes are present in the kidneys of infants and children with cirrhosis. Materials and Methods The kidneys from 24 autopsy cases of cirrhosis of the liver in infancy and childhood were studied. The tissue was fixed in 10% neutral formaldehyde solution and stained with hematoxylin and eosin. The PAS and Gomori trichrome stains were used for selected cases. The slides were examined by light microscopy. The clinical record and autopsy protocol of each case were reviewed. The patients (Table) ranged in age from 6 hours to 15 years. There were 11 boys and 13 girls. Seventeen were white, and seven, Negro. Alterations in renal function

Classification of primary malignant tumors of liver in infancy and childhood : K. Misugi, H. Okajima, N. Misugi and W. A. Newton, Jr. Cancer 20:1760–1771, October, 1967

Classification of primary malignant tumors of liver in infancy and childhood : K. Misugi, H. Okajima, N. Misugi and W. A. Newton, Jr. Cancer 20:1760–1771, October, 1967