Infantile hemangioendothelioma with increased serum alpha-fetoprotein / 대한간학회지

No abstract available.

A Case of Infantile Hemangioendothelioma of the Liver Treated with Hepatic Embolization and Lobectomy / 소아과

Infantile hemangioendothelioma (IHE) of the liver is the most common vascular tumor in infants before the age of 6 months. It is a histologically benign tumor with potentially life-threatening complications. The clinical manifestations are variable, ranging from asymptomatic forms to intractable high-output heart failure. In addition, abdominal mass, intraperitoneal hemorrhage due to rupture of mass, respiratory distress, hematologic abnormalities and jaundice can occur. Diagnostic work-up is through doppler ultrasound sonography, computed tomography scan, magnetic resonance imaging and angiography. Treatment consists of medical treatment, interventional therapy, surgical resection and liver transplantation. We experienced symptomatic IHE in a premature neonate who presented with high output heart failure and respiratory distress. Initial medical treatment and steroid therapy failed to improve his condition. Coil embolization of left hepatic artery resulted in improvement of respiratory symptoms. However, a left lobectomy was performed because the mass size was not decreased with development of collateral vessels. The infant was well, after a successful discharge from the hospital.

Giant Neonatal Hemangioendothelioma of the Liver Diagnosed by 99mTc-RBC Scintigraphy

Infantile hemangioendothelioma (IHE) is a benign tumor of the liver composed of anastomosing vascular channels lined by plump endothelial cells. The major clinical findings of IHE are abdominal mass, hepatomegaly, cutaneous hemangioma, congestive heart failure, anemia and disseminated intravascular coagulopathy. Precise diagnosis of IHE is crucial because medical therapies using steroid and/or interferon can be tried unless there are grave compressive symptoms. Along with CT scan and MRI studies, scintigraphic evaluation with 99mTc-RBC offers an accurate method of identification of these lesions, and allows differentiation from other common primary or secondary hepatic masses. We report two cases of giant IHE of the liver those were diagnosed with 99mTc- RBC scan and confirmed with pathologic evaluation after surgical removal.

Two Cases of Infantile Hemangioendothelioma of the Liver in Neonates

Infantile hemangioendothelioma(IHE) of the liver is a rare benign vascular tumor that presents most commonly in infants before the age of 6 months. IHE presents as abdominal mass, cutaneous hemangiomas, unexplained jaundice, bleeding disorders, or congestive heart failure. Death often results from congestive heart failure despite appropriate treatment with digoxin and diuretics. IHE also is associated with Kasabach-Merritt syndrome, anemia, intraperitoneal hemorrhage secondary to rupture, consumptive coagulopathy and vascular malformation involving brain, skin, gut, and other organs. Although children with asymptomatic lesions may experience spontaneous regression within a year, symptomatic lesions shoud be treated aggressively because this disease can progress rapidly and may be fatal. Treatment options are divided into medical treatment, interventional therapy including embolization, and surgical resection. Corticosteroid may hasten involution by inhibiting proliferation of endothelial and smooth muscle cells, and this trial is warranted in most cases before invasive procedures are used. If steroid therapy is unsuccessful, early definitive treatment using embolization or ligation of the hepatic artery, resectional surgery, and orthotopic liver transplantation shoud be considered. We experienced symptomatic IHE in two neonates. In the first case, she showed respiratory failure and consumptive coagulopathy, and symptoms were aggravated despite steroid therapy, so a left lobectomy was performed. In the second case, he presented high output cardiac failure, and was successfully treated by the coil embolization of left hepatic artery. This coil embolization of hepatic artery for treating IHE was the first case in Korea we know of.

Infantile Hemangioendothelioma of the Liver: Brief case report

Infantile hemangioendothelioma of the liver is a common vascular tumor in infancy. The tumor is usually multinodular or diffuse and classified into two types. We present a case of infantile hemangioendothelioma of the liver, which predominantly consists of type 2. A 4-month-old female was admitted for an evaulation of an abdominal distension. A CT scan of the liver showed a multinodular mass. The right lobectomy was done. Grossly, the mass consisted of round nodules ranging from 2cm to 5cm in diameter. Microscopically, the tumor revealed proliferation of small vascular channels lined by endothelial cells. Bizarre cells and mitotic cells were frequently noted. Vesicular nuclei and multilayering of the endothelial cells were also noted.

Infantile Hemangioendothelioma of the Liver: A histological and immunohistochemicalstudy of 4 cases

A pathological study was performed on four cases of infantile hemangioendothelioma of the liver. All the patients were between the age of 1 -5 months and the tumors were typical hemangio-endotheliomas, type 1. The tumors were composed basically of two components; the endothelial cell proliferation and the myxoid matrix. The endothelial cells were cytologically innocuous and formed vascular channels of varying sizes and shapes from capillary to sinusoidal and cavernous vessels. Fibrosis of the matrix, albeit not a major component of the tumor, was found particularly near the center. Immunohistochemically, CD31 was expressed strongly in almost all endothelial cells, in contrast to the stain for von Willebrand factor which was only focally and weakly positive. Alpha-fetoprotein was expressed in hepatocytes within the tumor or in hepatocytes around the tumor. Intratumoral bile duct structures were located mainly at peripheral portion. The results indicated that the type I infantile hemangioendothelioma is a tumor of endothelial cells and myxoid stroma, and that the endothelial cells undergo gradual maturation to form sinusoidal and cavernous vessels in accordance with gradual fibrosis of the myxoid stroma.