Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors have encountered benign liver masses as frequently as malignant lesions in children with hepatomegaly. Lesions studied included abscesses, cavernous hemangioma/hemangioendothelioma, adenoma of glycogen storage disease, choledochal cysts, focal nodular hyperplasia, cystic hepatoblastoma, and hamartoma. An integrated imaging protocol involving ultrasound, computed tomography, and scintigraphy proved to be more helpful than any one modality in establishing the benign or malignant nature of a hepatic neoplasm and the type of tumor, which is of particular importance when surgical exploration and/or biopsy is contraindicated.
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PMID:Integrated imaging of hepatic tumors in childhood. Part II: Benign lesions (congenital, reparative, and inflammatory). 388 Jun 15

Ultrasonography was useful in preoperative investigation of primary hepatic tumors in nine children, aged 4 weeks to 4 years. In all patients, the hepatic origin and the solid or mixed echogenic nature of the tumor was determined. In two children (one with hemangioendothelioma, one with cystic mesenchymoma) the ultrasonographic findings were even more specific when correlated with angiography and/or histology. Ultrasonography in children with hepatomegaly should be performed as a screening procedure before angiography, to determine if enlargement of the liver is of tumorous origin. For follow-up, ultrasonography should be preferred over radionuclide imaging because it does not require the use of ionizing energy.
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PMID:Ultrasonography in primary hepatic tumors in early childhood. 624 98

Infantile hepatic hemangioendothelioma is a rare tumor of infancy, sometimes associated with cutaneous hemangiomatosis. It is clinically evident within the first six months after birth and can be life threatening because of heart failure, intraperitoneal hemorrhage or thrombocytopenia. In less severe forms spontaneous regression has been described. Current treatment may be surgical ligation of the hepatic artery, or pharmacological therapy with corticosteroids or radiotherapy. A 4-month infant is described, admitted with acute heart failure and huge hepatomegaly. Since a surgical approach was not possible and corticosteroid therapy failed to achieve the expected effect, radiotherapy was given with excellent results.
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PMID:Infantile hepatic hemangioendothelioma treated by radiotherapy. 662 93

A case of hepatic hemangioendothelioma in an infant of 2 1/2 months of age is presented. This case showed clinical features of this malformation: cardiac failure, severe hepatomegaly and cutaneous hemangiomata. The cardiac failure was complicated by the presence of an atrial septal defect. This symptomatology persisted after the surgical closure of the cardiac defect. The control of cardiac failure was only possible by using high doses of metil-prednisolone (2 mg/kg/day). The scheme of management in the treatment with steroids was revised, and the good results obtained in this case and in other series recorded. The use of surgical techniques (hepatic artery ligation or lobectomy), and radiotherapy, is relegated to a secondary plane by these findings.
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PMID:[Hepatic hemangioendothelioma with interauricular communication]. 662 71

The distinctive sonographic appearance of benign vascular tumors of the liver (one hemangioendothelioma and two mesenchymal hamartomas) in three infants is presented. Each child had hepatomegaly and congestive heart failure, and all had confirmatory arteriography. The sonographic pattern of a complex mass, dilated proximal aorta, and enlarged nutritive and draining vascular structures should alert the radiologist and clinician to this diagnosis.
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PMID:Benign vascular tumors of the liver in infants: sonographic appearance. 697 23

The infantile hemangioendothelioma of the liver is a rare benign vascular tumor that accounts for some 10% of all primary hepatic masses. Two cases were seen at the Department of Pediatric Surgery of Vicenza's Hospital in the last ten years. Both of patients were females and presented in neonatal period asymptomatic. The first patient was found to have hepatomegaly by pediatrician at six weeks of age, the second was an incidental finding following ultrasound neonatal screening. The Authors outline some difficulties met with differential diagnosis between benign versus malignant hepatic tumors. Explorative laparotomy was considered necessary in both the patients. Liver biopsy was the only surgical procedure performed in the nonresectable tumor. Treatment with corticosteroids finally proved to be successful to determine progressive involution of the tumor. In the resectable form, left hepatic lobectomy was successfully performed because of the progressive size increase of the tumor.
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PMID:[Hemangioendothelioma of the liver in the newborn: description of 2 cases]. 819 23

Infantile hemangioendothelioma is a benign tumor of the liver composed of anastomosing vascular channels lined by plump endothelial cells. At initial presentation, most patients are 6 months of age or younger and have hepatomegaly or an abdominal mass. Congestive heart failure, bleeding, anemia, jaundice, and cutaneous or visceral hemangiomas may also be present. Grossly, the lesions are usually well circumscribed and may be focal, multifocal, or diffuse. Large solitary lesions are often associated with central hemorrhage or necrosis. Radiography reveals a mass that is occasionally calcified. Angiography reveals hypervascular lesions, often with arteriovenous shunting. A solid lesion with variable echotexture is noted at ultrasound. Computed tomography typically shows a low-attenuation solid lesion with peripheral enhancement. Central enhancement is often lacking except in smaller lesions. At magnetic resonance imaging performed with T2-weighted pulse sequences, the lesions usually have high signal intensity. Spontaneous regression of the tumor occurs, although patients may die of associated conditions.
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PMID:From the archives of the AFIP. Infantile hemangioendothelioma of the liver revisited. 831 72

The study documents the immunohistochemical features of a case of infantile hemangioendothelioma (IHE) of the liver, which was found incidentally at autopsy in a 44-day-old girl. A precardial apical systolic murmur and hepatomegaly were found on day 4 of life. The tumor was multifocal and histologically composed of vascular channels lined by endothelial cells that were positive for von Willebrand factor, CD31, vimentin, and Ulex europaeus agglutinin 1, and that were invested in a continuous basement membrane (BM) on the antiluminal border. The endothelial cells, especially in the region of intravascular buds, showed intracytoplasmic synthesis of BM components (laminin and collagen IV). Underlying the endothelial cells were cells with cytoplasm that was positive for alpha-smooth muscle actin and antimuscle actin and negative for desmin, and that were enveloped with BM. The immunophenotype, appearance, and location of these cells are characteristic of pericytes. We found neither signs of endocrine secretion nor hepatitis B virus in the tumor tissue. The appearance of this tumor in the neonatal period supports a fetal origin of IHE.
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PMID:Infantile hemangioendothelioma of the liver in a neonate. Immunohistochemical observations. 866 36

Multiple hepatic hemangioendothelioma are vascular lesions of the liver that generally appear in the infancy with hepatomegaly, high output congestive heart failure and cutaneous hemangiomas. Many plans for management (steroid, radiation, hepatic artery ligation, embolization, cyclophosphamide) have been proposed. We report a case in two months old boy of hepatic hemangioendothelioma with arteriovenous shunts and heart congestive failure, successfully treated with steroid.
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PMID:[Efficacy of steroid therapy in a case of hepatic hemangioendothelioma in childhood]. 876 89

A case of infantile hemangioendothelioma of the liver is reported in a 18 day-old male neonate. The neonate presented with CHF. He had hepatomegaly, but lacked the third component of the classical triad i.e. cutaneous hemangiomas. The S.A.F.P levels were raised to 920 ng/ml, though rise is rare in infantile hemangioendothelioma and led to the clinical diagnosis of hepatoblastoma. A left hepatic resection was performed, the histology showed a type I pattern of the tumor. This is known to be associated with a good prognosis. The baby is well post resection for a follow up period of six months.
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PMID:Hepatic hemangioendothelioma of infancy with congestive cardiac failure--report of a case. 877 23


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