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 case of a 19 yr-old female with abdominal pain, jaundice, and hepatomegaly is reported. A large mass replacing the left hepatic lobe, detected by scintigraphic and angiographic studies and also by means of ultrasound and transhepatic radio-opaque filling, proved to be cystic. A left hepatic lobectomy was performed. Pathologic examination revealed a mesenchymal hamartoma, a rather rare event in view of the patient's age. Prompt recovery followed surgery, and the patient is well and symptomless 1 yr after operation.
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PMID:Mesenchymal hamartoma of the liver. 43 45

Mesenchymal hamartoma of the liver occurs almost exclusively in infancy and childhood, with approximately 140 total cases reported. We report the experience with 18 patients at the Childrens Hospital of Los Angeles (Calif) during the past 35 years. The charts of all patients with mesenchymal hamartoma were retrospectively reviewed. The mean age at presentation was 16 months. Thirteen patients were symptomatic, presenting with increasing abdominal distention. Physical examination revealed an abdominal mass or hepatomegaly. Ultrasonography and computed tomography were the most useful diagnostic tests. Fourteen patients underwent resection; 9 underwent hepatic resection and 5 underwent excision of the tumor only. One patient had marsupialization, 1 underwent a biopsy only, and 2 died of unrelated causes and the hamartoma was found incidentally at autopsy. In all instances, a large cystic mass with well-demarcated margins was found. Three patients were unavailable for follow-up and 13 patients were alive and well 1 month to 24 years (mean, 5 years) after diagnosis. Recurrence or malignant transformation was not noted. A presumptive diagnosis can be made preoperatively by normal laboratory values and a combination of ultrasonography and computed tomography. We recommend excision of the tumor in all patients once the diagnosis is made, with the expectation of complete recovery.
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PMID:Mesenchymal hamartoma of the liver. A 35-year review. 233 Dec 17

A 28-year-old black woman presented with increasing abdominal girth and gross hepatomegaly. Preoperative investigations demonstrated an enlarging vascular tumor involving the right lobe of the liver. A right hepatic trisegmentectomy was performed, and histologic examination of the tissue revealed a mesenchymal hamartoma, a tumor usually presenting in early childhood. This case is unique because the patient is 22 years beyond the average presenting age, and is the oldest patient reported.
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PMID:Mesenchymal hamartoma of the liver: report of a case in a 28-year-old. 340 88

An 8 year old girl presented with gross hepatomegaly, anaemia and pyrexia unresponsive to antibiotics. At laparotomy a cystic mass 19 cm in diameter was found arising from the liver. The cyst contained 2.4 litres of changed blood and an abscess cavity filled with thick pus was present in the cyst wall. Histological examination showed the appearances of a mesenchymal hamartoma of the liver, a rare diagnosis at this age. This case is unusual as mesenchymal hamartoma has not been previously reported presenting with pyrexia and anaemia as well as hepatomegaly.
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PMID:Mesenchymal hamartoma of the liver--report of an unusual case. 377 8

The case of a 32-year-old female with asymptomatic hepatomegaly due to a large cystic liver mass is described. Pathological examination after total excision revealed a benign hepatic hamartoma. Benign hepatic hamartoma is a rare cause of a cystic liver mass, usually occurring below the age of 5 years. The computed tomography findings included 'daughter cysts' and it is emphasised that these must not be regarded as pathognomonic of hydatid disease.
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PMID:The computed tomographic appearances of benign hepatic hamartoma. 381 57

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

In a newborn infant with heart failure and hepatomegaly the radiological findings suggested hepatic haemangioendothelioma, but subsequently the tumour proved to be a hamartoma.
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PMID:Hepatic hamartoma in a newborn. 736 56

An infant with persistent hyperinsulinemic hypoglycemia, diffuse nesidioblastosis, and mixed hamartoma of the liver (MHL), in addition to demonstrating clinical, pathologic, and molecular manifestations of Beckwith-Wiedemann syndrome (BWS), is the subject of this report. H19 methylation assay and allelic expression analysis for insulin-like growth factor 2 (IGF2) indicated that the patient was mosaic for paternal isodisomic cells and normal cells in lung tissue, nontumoral liver tissue, tissue from the MHL, and pancreatic tissue. We propose that abundant IGF2 expression during development due to paternal isodisomy resulted in hepatomegaly and islet cell hyperplasia, which led to nesidioblastosis. MHL, by contrast, may have resulted from a decrease in disomic cells, compared with nontumoral liver tissue, which showed an increase in disomic cells. Thus, somatic mosaicism may result in unbalanced tissue growth, which may contribute to the formation of MHL in BWS.
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PMID:Nesidioblastosis and mixed hamartoma of the liver in Beckwith-Wiedemann syndrome: case study including analysis of H19 methylation and insulin-like growth factor 2 genotyping and imprinting. 1144 40

We report a hepatic tumor in an adolescent that does not fit into any of the described categories of liver tumors. The patient presented with hepatomegaly, abdominal pain, and normal liver function test; the tumor was cystic in imaging studies. The resected specimen, result of a partial hepatectomy, measured 21 cm and was multicystic with solid areas. Microscopically, the cysts were lined by a mucous-producing or intestinal-type epithelium, associated with smooth muscle and small mucous-producing glands. The solid component contained fibrous and adipose tissue, smooth muscle and thick-walled vessels. Aneuploidy was demonstrated by flow cytometry. We interpreted the tumor as having features of a mesenchymal hamartoma and congenital solitary nonparasitic cyst. It is conceivable that the lesions originated with small peribiliary glands with dilatation and intestinal metaplasia.
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PMID:Unusual hepatic tumor with features of mesenchymal hamartoma and congenital solitary nonparasitic cyst. 1267 72

Mesenchymal hamartoma of the liver is a rare congenital disorder of biliary tract development. During the necropsy of a late-term equine fetus, a markedly enlarged liver of more than two times normal weight was found. Light microscopic review revealed that the normal hepatic parenchyma had been obliterated, replaced, and expanded by abnormal bile ducts surrounded by abundant, myxoid stroma. The lesion was diagnosed as a mesenchymal hamartoma. Small portions of the liver had bridging septa of fibrosis and proliferations of small-caliber abnormal bile ducts, resembling another congenital biliary abnormality termed congenital hepatic fibrosis.
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PMID:Mesenchymal hamartoma of the liver in a late-term equine fetus. 1719 32


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