Cases reported "Liver Diseases"

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1/46. New sonographic appearance of hepatic mesenchymal hamartoma in childhood.

    We present clinical, radiographic, and sonographic findings in 3 children with hepatic mesenchymal hamartoma, a rare benign tumor of childhood. The presence of round hyperechoic parietal nodules within the cystic spaces of the hamartomas is a new sonographic finding.
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2/46. prenatal diagnosis of hepatic mesenchymal hamartoma.

    A case of hepatic mesenchymal hamartoma diagnosed prenatally with ultrasound and confirmed histologically post-delivery is presented. Although histologically benign, this lesion resulted in fetal demise secondary to congestive cardiac failure in the third trimester. The development of non-immune hydrops in association with a fetal hepatic mesenchymal hamartoma is a poor prognostic sign for perinatal survival.
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3/46. Mesenchymal hamartoma of the liver--new insight into histogenesis.

    BACKGROUND/PURPOSE: Mesenchymal hamartoma (MH) of the liver is thought to develop from the ductal plates of the prenatal liver. This immunohistochemical study was performed to gain insight into the pathophysiology of its development. methods: Specimens from four MHs with adjacent liver, in one case from a biopsy and from the resected lesion after 6 years follow-up, were investigated with immunostaining on cryostatsectionswith antibodies against cytokeratins, vimentin, desmin and alpha-actin, as well as von willebrand factor (factor viii), fibroblast growth factor (FGF) receptors, FGF-1 (acidic FGF), FGF-2 (basic FGF), and the proliferation-associated Ki67 antigen. RESULTS: Fibrous tissue of MH stained positive not only for vimentin, but also for desmin and alpha-actin, whereas cytokeratins and factor viii showed specific staining in biliary cysts and endothelial cells, respectively. All mesenchymal cells expressed proteins of the FGF receptor family. Although FGF-1 was only scarcely detectable, there was an accumulation of FGF-2 in borderline areas of liver to MH. Multiple Ki67-positive mesenchymal cells could be identified in these regions in all three MHs. However, we could not detect any proliferative activity in the MHs after follow-up. CONCLUSIONS: The proliferative process in MH is still active during early childhood. FGF-2 may have a role in promoting this process. The positivity for desmin and alpha-actin of the lesions suggests that fat-storing (Ito) cells of the immature liver may be involved in the development of MH.
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4/46. Malignant transformation of mesenchymal hamartoma of the liver: case report and review of the literature.

    Here the first case in the literature of both mesenchymal hamartoma and malignant mesenchymoma occurring in a 6-year-old male child, at different times and at different sites in the liver, and also the possible malignant transformation of a mesenchymal hamartoma is reported. The tumor developed from a lesion in the right lobe that was overlooked initially during a left lateral segmentectomy at 18 months of age for a mesenchymal hamartoma. Malignant mesenchymoma is a rare and aggressive tumor. The origin of this tumor is not well understood. There has been no direct support to the hypothesis that malignant mesenchymoma may be the malignant counterpart of mesenchymal hamartoma. The authors provide clinical and histopathologic evidence in our case that suggests the possibility of malignant mesenchymoma arising from a mesenchymal hamartoma. This case emphasizes the need for complete removal of mesenchymal hamartoma and the need for long-term follow-up to detect multifocal lesion or malignant transformation.
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5/46. Hepatic mesenchymal hamartoma associated with mesenchymal stem villous hyperplasia of the placenta.

    A rare case of prenatally diagnosed hepatic mesenchymal hamartoma associated with mesenchymal stem villous hyperplasia of the placenta is presented and the literature reviewed. The placenta was noticed to have multiple cysts at 16 weeks' gestation and elevated maternal serum alpha-fetoprotein was present. Cystic liver masses were first detected in the fetus at 34 weeks' gestation. The liver mass showed a progressive enlargement during the third trimester and after birth, necessitating extended left hepatic trisegmentectomy at 3 months of age. Histological examination established the diagnosis of mesenchymal hamartoma of the liver and mesenchymal stem villous hyperplasia of the placenta. This is the first report of this association in which both lesions were diagnosed in utero and confirmed by histology after delivery. The likely pathogenesis of these strikingly similar lesions is discussed.
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6/46. Fine-needle aspiration cytology of mesenchymal hamartoma of the liver.

    The cytologic appearance of mesenchymal hepatic hamartoma in a 2-yr-old boy is described. Smears disclosed small groups and isolated, benign-appearing spindle cells admixed with scarce amounts of myxoid stroma and normal ductal cells and hepatocytes. Although the findings were nonspecific, cytology may rule out many other diagnostic possibilities and increases the preoperative capacity of clinical and image studies, leading to a more rational therapeutic decision.
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7/46. Mesenchymal hamartoma of the liver.

    We present a multiseptated mesenchymal hamartoma of the liver in a 10-year-old male patient, a rare benign tumor of childhood. The characteristic ultrasound and CT appearances of this unusual tumor are reviewed. A single septal calcification associated with this tumor was demonstrated, an association which has not previously been reported. The differential diagnosis for cystic liver lesions is discussed in detail.
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8/46. Postpartum follow-up of hepatic calcification detected by prenatal ultrasound.

    The causes of parenchymal hepatic calcification are mostly transplacental infection of TORCH complex, ischaemic necrosis, or tumours of foetal liver including haemangioma, hamartoma, teratoma, hepatoma, and hemangioendothelioma. Vascular pathologies like hepatic artery aneurysm, haematoma, calcified thromboemboli of portal vein and hepatic veins can also cause hepatic calcification. We present a case of hepatic calcification which was first diagnosed by prenatal ultrasound. In the postpartum follow-up, we observed that the calcifications had decreased in number and size. The causes and results of hepatic calcification or underlying disease are discussed with analysis of the literature.
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9/46. Mesenchymal hamartoma of the liver in the adult: association with distinct clinical features and histological changes.

    Mesenchymal hamartoma of the liver (MHL) is an uncommon mass lesion composed of architecturally abnormal bile ducts in an uncommitted myxoid stroma. Most MHL are diagnosed in childhood. More than 50% of cases are seen in the first year of life, although a few cases have been previously reported in adults. The spectrum of pathological findings in the cases presenting in adults, including differences in comparison with MHL in children, has not been fully characterized. In this report, we describe 3 cases of MHL in patients 46, 63, and 66 years of age. Each of the patients was a woman who had a solid or multicystic hepatic mass ranging from 5 to 24 cm in maximum dimension. In contrast to the childhood cases, the stromal component was fibrotic with areas of dense hyalinization and only focal myxoid areas. In 1 case, the mesenchymal component was the predominant feature of the lesion, with only occasional ductal elements identified with thorough tissue sampling. Immunohistochemical analysis with a panel of antibodies showed that, as in normal bile ducts, the ductal structures within the lesion were immunoreactive for cytokeratin 7 and negative for cytokeratin 20. The stroma was composed of a prominent population of fibroblasts and myofibroblasts that were positive for smooth muscle actin and vimentin. Analysis of the current findings, together with the previous case reports, shows that in contrast to MHL in children, this lesion in adults is found more commonly in women who present with abdominal pain. Recognition of the clinicopathologic differences between adult and pediatric cases will facilitate accurate diagnosis of this uncommon lesion.
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10/46. Unusual hepatic tumor with features of mesenchymal hamartoma and congenital solitary nonparasitic cyst.

    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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