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)

Of 16 infants with infantile hepatic hemangioendothelioma, 14 (88%) presented before age 3 months. For seven cases (44%), the diagnosis was suspected from antenatal ultrasonographic findings. Two (13%) presented with asymptomatic hepatomegaly. The most common presenting features were high-output cardiac failure in 11 (69%), consumptive coagulopathy in 12 (75%), and anemia in 12(75%). Sixty-three percent of the children had associated cutaneous hemangiomata, and disseminated hemangiomatosis was noted in two (13%). Medical measures were effective in stabilizing seven (44%) cases with high-output congestive cardiac failure and/or consumptive coagulopathy. Partial right hepatectomy was successful for four patients; the only death occurred in a newborn, after intraperitoneal rupture of the hepatic hemangioma. Embolization was used in two children to induce involution. Spontaneous involution occurred in two patients. Initially, hepatic hemangiomas should be treated conservatively, with surgery reserved for intractable cardiac failure and/or refractory consumptive coagulopathy.
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PMID:Infantile hepatic hemangioendothelioma: the role of surgery. 878 79

A female infant, born at the gestational age of 29 weeks with a birthweight of 1,350 gm, developed progressive hepatomegaly at 10 days of age. Congestive heart failure gradually developed, and hepatic hemangioendothelioma was diagnosed at 1 month of age by open biopsy. Due to rapid enlargement of the tumor and progressive heart failure, steroid therapy was given from 36 days of age, including methylprednisolone 15 mg/kg/day for 3 days and 10 mg/kg/day for 4 days, then prednisolone 4 mg/kg/day for 20 days followed by tapering till 74 days of age. The tumor regressed gradually and was not detectable by sonography at 33 months of age. We suggest that, even in very-low-birthweight infants, the prognosis of hepatic hemangioendothelioma may be improved with aggressive therapy when symptoms develop during tumor progression, and that steroid should be the initial treatment.
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PMID:Hepatic hemangioendothelioma: successful treatment with steroid in a very-low-birth-weight infant. 893 13

Infantile hemangioendothelioma is the most common vascular tumor in infancy. A three-month-old infant was admitted to the hospital for hepatomegaly. Abdominal ultrasonography, a CT-scan, and MRI studies demonstrated bilateral diffuse hepatic nodules, which were characteristic of hepatic hemangioendothelioma. A highly elevated alpha-fetoprotein (AFP) level misled us to an impression of hepatoblastoma, but the pathology report through an open biopsy disclosed a liver hemangioendothelioma. The patient responded to methylprednisolone therapy. A follow-up sonogram revealed regression of the hepatic masses. This case emphasizes that an elevated AFP level of up to 400 ng/ml is normally found in some neonates until two months of age. Careful interpretation of this value is very important, especially when it is associated with a hepatic tumor. Herein, we present a case of infantile hemangioendothelioma in a three-month-old boy with a highly elevated serum AFP.
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PMID:Infantile hemangioendothelioma with a highly elevated serum alpha-fetoprotein level. 963 27

Imaging investigations and other findings observed in a term infant with a multicentric hepatic hemangioendothelioma, admitted to the Intensive Care Unit at the age of 13 days because of non specified feeding difficulties and dyspnoea, are presented. Physical examination revealed cardiac bruit and congestive heart failure with marked hepatomegaly; in addition there were multiple small skin hemangiomas. Echocardiography was negative, abdominal sonography showed multiple round lesions of mixed echogenicity in the liver, large vascular channels, a right hepatic artery and hepatic veins enlarged, a caliber of the aorta below the level of the superior mesenteric artery reduced. The infant was additionally investigated by whole-body scintigraphy with 99mTc-labeled red blood cells to determine the possibility of coexistence of other visceral hemangiomas and by MR, in which the tumor manifested as multiple well-circumscribed space-occupying nodules of high signal intensity on T2-weighted images with evidence of fast flow. The baby underwent furosemide and steroid therapy: serial two-dimensional US scans showed change in echogenicity, responding to therapy. Doppler sonography has proven to be also very useful in the monitoring therapy determining changes in flow pattern and velocity at the level of hepatic, cerebral and renal vessels: before therapy we observed a reduction of the diastolic flow until the zero line through the internal carotid artery and renal artery with an increase of the Resistance Index. It means that this important component can be compromised in the presence of a hepatic hemangioendothelioma.
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PMID:[A case of congenital hepatic hemangioendothelioma treated with prednisone: the echographic changes and Doppler study]. 967 4

We report a case of a 2 1/2-year-old girl presenting with abdominal pain, fever, vomiting, and hepatomegaly. In spite of the unusual age at presentation, dynamic gadolinium-enhanced MR findings, which have not been previously illustrated, proved to be highly specific for the diagnosis of infantile hepatic hemangioendothelioma because of the characteristic enhancement pattern.
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PMID:Dynamic gadolinium-enhanced MR findings in infantile hepatic hemangioendothelioma. 975 3

We describe a case of hepatic hemangioendothelioma that was first suspected based on prenatal sonographic findings at 19 weeks' menstrual age. At 16 weeks, the patient presented with a markedly elevated maternal serum alpha-fetoprotein level. Serial sonographic examinations revealed that the fetus had cardiomegaly, hepatomegaly with a hepatic mass and dilated intrahepatic vessels, a single umbilical artery, and a placental chorioangioma. Arteriovenous shunting within the hepatic mass was seen using color Doppler and pulsed Doppler sonography. An enlarged artery arising from the abdominal aorta supplying the mass was demonstrated. Postnatal physical examination and radiologic studies supported the diagnosis of hepatic hemangioendothelioma. The evolution in the sonographic appearance of this hepatic lesion in utero over a 17-week period is described.
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PMID:Hepatic hemangioendothelioma: prenatal sonographic findings and evolution of the lesion. 1080 6

Hepatic hemangioendothelioma (HE) is a tumor that presents in infancy and toddler. It manifests hepatomegaly, abdominal mass, jaundice, abdominal distention, or high output cardiac failure. We reviewed patients with HE in our hospital in the past 15 years (from July 1986 to June 2001). The diagnosis was made by the histology specimen or various imaging studies. There were thirteen patients (9 males, 4 females) enrolled in our study. Their ages ranged from neonate to 2 years old. The common clinical manifestations included abdominal distention (53%), congestive heart failure (38.5%), abdominal mass (30.8%), jaundice (30.8%), and skin hemangioma (23.1%). Nine patients had serum alanine aminotransferase examination and were abnormal in 2. Anemia was noted in 7 of 13 (53.8%) patients, thrombocytopenia and hyperconsumptive coagulopathy were found in 4 and 5 patients, respectively. Serum alpha-fetoprotein was elevated in 4 of 7 patients. Abdominal ultrasonography (n = 13) showed heterogeneous and hypoechoic lesions in the liver. Computed tomography (n = 11) revealed central hypointensity with peripheral enhancement after contrast of the liver masses. Magnetic resonance imaging studies of the hepatic masses (n = 3) showed decreased signal intensity on T1 images and high signal intensity on T2. Most patients were treated with steroid. Other management included interferon, chemotherapy, embolization and/or surgery. Four patients were managed conservatively. Among the other nine patients, four patients died of sepsis, hepatic failure, disseminated intravascular coagulopathy or tumor rupture with hemorrhagic shock. HE appears to be a histologically benign tumor but may have a poor outcome because of complications. For its management, steroid is a first-line medication. Other methods of treatment were interferon, hepatic artery embolization, chemotherapy and surgery. Long term follow up is needed for the evaluation of treatment response.
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PMID:Hepatic hemangioendothelioma in children: analysis of thirteen cases. 1280 Mar 77

The authors describe a 10-week-old girl with infantile hepatic hemangioendothelioma who initially presented with difficulty feeding, hepatomegaly, and multiple hemangiomas of the skin. Six weeks of steroid therapy and 2 weeks of chemotherapy failed to produce clinical improvement. The patient underwent split liver transplantation. A definitive diagnosis of hemangioendothelioma type II was made. Imaging studies cannot differentiate between hemangioendothelioma and angiosarcoma. Treatment modalities for this condition remain unclear. The patient continues to do well.
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PMID:Congenital infantile hepatic hemangioendothelioma type II treated with orthotopic liver transplantation. 1476 4

Epithelioid hemangioendothelioma and angiosarcoma of the liver are rare neoplasms of vascular origin. They can present with nonspecific symptoms such as malaise and weight loss, as well as with liver-related symptoms such as abdominal pain, tender hepatomegaly and jaundice. Portal hypertension and rarely liver failure can occur. We hereby report two cases of fulminant hepatic failure that were eventually diagnosed with epithelioid hemangioendothelioma and angiosarcoma of the liver.
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PMID:Unusual presentation of hepatic vascular tumors as fulminant hepatic failure. 1637 9

Hepatic epithelioid hemangiendothelioma is a rare vascular tumor. The clinical course is unpredictable and different treatment modalities are offered depending on the patients condition. Orthotopic liver tranplantation is the choice of treatment in diffuse cases without metastases. A 32 year old woman was admitted to hospital with multiple mass lesions diagnosed by ultrasonography of the liver. Physical examination was normal except for a painless hepatomegaly, and her biochemical tests were within the normal range. Computed tomographic scanning showed the presence of multiple lesions in both lobes, some of which were accompanied by a small degree of calcification. Although these findings were suggestive of hepatic epithelioid hemangioendothelioma, ultrasonographic guided fine needle aspiration biopsy failed to diagnose the exact nature of the lesions. The diagnosis of hepatic epithelioid hemangioendothelioma was confirmed by diagnostic laparotomy and immunohistochemical examination of the specimen by FVIII-RAg, CD34 and CD 31 markers. The patient was treated by orthotopic liver transplantation and had no evidence of tumor 18 months after transplantation. The problems in differential diagnosis and treatment options are discussed in this report of the first case of this rare tumor, treated by orthotopic liver transplantation in Turkey.
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PMID:Hepatic epithelioid hemangioendothelioma treated with orthotopic liver transplantation: a case report. 1637 10


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