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 study documents the immunohistochemical features of a case of infantile hemangioendothelioma (IHE) of the liver, which was found incidentally at autopsy in a 44-day-old girl. A precardial apical systolic murmur and hepatomegaly were found on day 4 of life. The tumor was multifocal and histologically composed of vascular channels lined by endothelial cells that were positive for von Willebrand factor, CD31, vimentin, and Ulex europaeus agglutinin 1, and that were invested in a continuous basement membrane (BM) on the antiluminal border. The endothelial cells, especially in the region of intravascular buds, showed intracytoplasmic synthesis of BM components (laminin and collagen IV). Underlying the endothelial cells were cells with cytoplasm that was positive for alpha-smooth muscle actin and antimuscle actin and negative for desmin, and that were enveloped with BM. The immunophenotype, appearance, and location of these cells are characteristic of pericytes. We found neither signs of endocrine secretion nor hepatitis B virus in the tumor tissue. The appearance of this tumor in the neonatal period supports a fetal origin of IHE.
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PMID:Infantile hemangioendothelioma of the liver in a neonate. Immunohistochemical observations. 866 36

Ewing sarcoma/primitive neuroectodermal tumor is a rare tumor of soft tissues of thoraco-pulmonary regions, pelvis and lower extremities. Involvement of visceral organs by primitive neuroectodermal tumor is even rarer, with the kidney being the most commonly involved organ. Involvement of the liver has been reported in the form of metastasis from other primary sources presenting as liver abscess. We report a 20-year-old lady presenting with massive hepatomegaly, with computed tomography scan evidence of diffuse hepatomegaly and a normal porta and intrahepatic biliary radicles. She subsequently underwent ultrasonography-guided true-cut needle biopsy of the liver. Histopathology of the liver revealed nests of small round blue tumor cells in the background of hepatocytes infiltrating the liver, which expressed Mic-2 and Fli-1, and were negative for cytokeratin, desmin, hepatocyte-specific antigen (OCHIE5), synaptophysin, chromogranin A and CD-20. Immunohistochemistry revealed CD-99-positive. Extensive search regarding any possible different site of involvement by the tumor was negative. The patient responded to a combination therapy of vincristine, adriamycin and cyclophosphamide alternating with ifosfamide and etoposide 3 weekly over 43 weeks and has been doing well even after 1 year of diagnosis. The clinical presentation, the macroscopic aspect, together with the histological pattern, the cytological characteristic and the cellular immunophenotype lead to the diagnosis of primary primitive neuroectodermal tumor of the liver which responded well to combination chemotherapy.
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PMID:Primitive neuroectodermal tumor of the liver: a case report. 1999 88