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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An infant presented with congenital hemihypertrophy, hepatomegaly, and a low thoracic paraspinal mass. Liver scan showed multiple defects in the uptake of radioisotope. Surgical exploration revealed multiple infantile hemangioendotheliomas of the liver and a paraspinal hemangioendothelioma. In review of 69 reported cases of infantile hemangioendothelioma of the liver and viscera, there has been no previous note of hemihypertrophy associated with this abnormality.
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PMID:Infantile hepatic hemangioendotheliomas associated with hemihypertrophy. 26 14

Hemangioendothelioma of the liver is a rare tumor. A case is reported of a middle-aged woman who presented with hepatomegaly and ascites, and who has been followed for over 12 years. The clinical features, pathological, radiographic, portal hemodynamic, and metabolic changes are presented.
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PMID:Hemangioendotheliomatosis of the liver. A 12-year follow-up. 83 Feb 30

Seven patients with hemangiomatosis of the liver are presented together with 100 cases reported in the literature. Six of seven patients survived; four with supportive treatment only. Follow-up is from 2 to 11 yr. Hepatomegaly, heart failure, and cutaneous angiomas constitute the classical clinical triad. Arteriography is the best method for diagnosis; biopsy should be avoided. Clinical features and histologic examination of these benign vascular tumors seem to confirm the theory that hemangioendothelioma and hemangioma represent only different developmental stages of one disease. These lesions can regress spontaneously if left alone. Despite contrary prevailing opinion, the authors recommend nonoperative treatment as the first choice of the clinician.
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PMID:Hemangiomatosis of the liver in infants. 111 83

The authors present 7 patients with hemangiomatosis of the liver together with about 100 cases reported in the literature. 6 of 7 patients survived; 4 with supportive treatment only. Follow-up is from 2 to 12 years. Hepatomegaly, heart failure and cutaneous angiomas constitute the classical clinical triad. Arteriography is the best method to diagnose this lesion, and biopsy can thus be avoided. Clinical features and histological examination of these benign vascular tumors seem to confirm the theory that hemangioendothelioma and hemangioma represent only two different developmental stages of one disease. These lesions can regress spontaneously if left alone. Despite contrary prevailing opinion the conservative treatment is recommended as the first choice of the clinician.
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PMID:[Hemangiomatosis of the liver in infants: Diagnosis and treatment]. 115 Apr 80

Two children are reported with hemangioendothelioma of the hepatobiliary system. One patient, a 7-month-old girl, presented with classic symptomatology of hepatomegaly and congestive heart failure, but did not have any cutaneous lesions. She was treated with systemic steroids and supportive therapy and did very well. The other patient, a 22-month-old boy, is the first patient reported with exclusive involvement of the extrahepatic biliary tree. His symptoms included hepatomegaly and obstructive jaundice. He was evaluated with an endoscopic retrograde cholangiopancreatogram and a transhepatic cholangiogram. He did very well after resection of the lesion and Roux-en-Y drainage of the proximal biliary tree and remains asymptomatic 2 years after surgery.
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PMID:Hemangioendothelioma of the hepatobiliary system: the classic and the unusual. 218 12

A case of infantile hemangioendothelioma of the liver presenting with congestive heart failure and massive hepatomegaly in a two-mth old baby is described. Serum alpha-fetoprotein was elevated in this patient and clinically the lesion might not be distinguishable from other, commoner hepatic tumours in children. Histologically the tumour showed a type II pattern as defined by Dehner. The significance of classifying this tumour into two histologic types is discussed. Only 3 cases reported in the English literature were studied by electron microscopy; two of them were type I while the other was a type II lesion. In our case the endothelial cells appeared ultrastructurally less differentiated and architecturally more disorganized than in type I lesions. Like the previous case, the basal lamina showed marked thickening and reduplication. The significance of these findings is discussed.
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PMID:Infantile hemangioendothelioma of the liver: ultrastructural study of a type II case. 243 5

A distinct syndrome in infantile hemangioendothelioma consist of high-output congestive heart failure, hepatomegaly and cutaneous hemangiomas. Early therapy aimed at reduction of the shunt is essential if life is to be saved once cardiac failure becomes manifest. We report a case of infantile hemangioendothelioma with massive A-V and P-V shunt successfully treated by transcatheter embolization.
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PMID:[Therapeutic embolization of infantile hemangioendothelioma of the liver]. 268 11

This report describes our experience with 16 infants and children with a mean age of 7 months and a median age of 6 weeks with hepatic hemangioendothelioma. Fifteen patients presented with hepatomegaly. Seven had congestive heart failure and four had associated cutaneous lesions. Although diagnosis was clinically evident in 15 of the 16 patients, arteriography and computerized tomography (CT) were diagnostic. A variety of treatment approaches were used including radiation, resection, systemic steroids, hepatic artery ligation, angiographic embolization, and various combinations of these modalities. The following information was gained from this experience. CT with enhancement is as specific a diagnostic tool as hepatic arteriography. Angiographic and CT appearances do not correlate with prognosis. Most patients can be treated successfully with steroids; those who do not respond should have other approaches tried such as embolization or ligation. The angiographic appearance determines whether embolization therapy is worthwhile, as we found that hemangioendotheliomatosis with portal as well as hepatic arterial supply will not respond to embolization. The survival rate in this series was 80%.
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PMID:Experience with hepatic hemangioendothelioma in infancy and childhood. 320 67

Hepatic hemangioma (hemangioendothelioma) is a benign vascular tumor with significant morbidity and mortality. Two newborn infants underwent partial hepatectomy for treatment of massive hepatic hemangioma, one with atrial septal defect and secondary congestive heart failure, and the other with symptomatic marked hepatomegaly. Cardiopulmonary bypass with induced deep hypothermic circulatory arrest was used in both patients in order to facilitate resection and prevent sudden massive blood loss. One infant also had closure of an atrial septal defect in addition to the partial hepatectomy. Both infants survived without complication and showed complete resolution of preoperative symptoms. This technique coupled with the use of fibrin glue is of value in infants with large hepatic hemangiomata and cardiac decompensation in the neonatal period.
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PMID:Hepatic hemangioma: resection using hypothermic circulatory arrest in the newborn. 323 62

The vascular origin lesions of the liver (capillary hemangioma/infantile hemangioendothelioma) that present in infancy or early childhood often have a typical clinical picture of hepatomegaly and congestive heart failure. These lesions rarely present as asymptomatic hepatomegaly, simulating a primary hepatic malignancy. These lesions may also simulate a primary or secondary hepatic malignancy on cross-sectional imaging or angiography. Scintigraphic evaluations with technetium-99m-labeled red blood cells offers an accurate method of identification of these lesions, and allows differentiation from other common primary or secondary hepatic masses in infancy or childhood. This scintigraphic method may also be used to follow these patients after medical, radiation, or embolization therapy. Experience with seven patients with these tumors is reported and compared with eight children with other primary or secondary liver tumors also evaluated by this method.
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PMID:Technetium-99m-labeled red blood cells in the evaluation of hemangiomas of the liver in infants and children. 362 94


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