Hepatic hemangioendothelioma of infancy associated with elevated alpha fetoprotein and catecholamine by-products.
Five cases of hepatic hemangioendothelioma (HH) were seen in infants ranging from 2 days to 5 months of age. The cases were studied by clinical chemistry, immunohistochemical staining, and electron microscopic techniques. Serum alpha-fetoprotein (AFP) levels were elevated in each of the four patients in whom levels were obtained. The highest elevation was noted in a 2-day-old infant with a diffuse, unresectable lesion involving the right lobe. Two patients who underwent complete resections of their solitary HH had normalization of their AFP levels. This study suggests that AFP levels in patients with HH are closely related to the patients' age, with the youngest patient having the highest levels of AFP. Catecholamine by-products (VMA and HVA) were elevated in one of four patients in whom levels were obtained, this infant was 3 months old at the time of presentation. She also had an elevated AFP level, a diffuse unresectable lesion involving the entire liver, multiple pulmonary nodules, and cutaneous hemangiomas. Immunohistochemical study failed to demonstrate the source of AFP or the catecholamine by-products in the tumor of this patient, suggesting that the source of catecholamine by-products could be stress-induced catecholamine secretion.
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Department of Pathology, Wishard Memorial Hospital, Indiana University Medical Center, Indianapolis 46202.
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Mirkin
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D000941:Antigens / Q000378:metabolism; D005169:Factor VIII / Q000276:immunology / Q000378:metabolism; D005260:Female; D005500:Follow-Up Studies; D006390:Hemangioendothelioma / Q000378:metabolism* / Q000648:ultrastructure; D006719:Homovanillic Acid / Q000378:metabolism*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D037102:Lectins / Q000378:metabolism; D008113:Liver Neoplasms / Q000378:metabolism* / Q000648:ultrastructure; D008297:Male; D037121:Plant Lectins; D014642:Vanilmandelic Acid / Q000378:metabolism*; D014746:Vimentin / Q000378:metabolism; D000509:alpha-Fetoproteins / Q000378:metabolism*; D014841:von Willebrand Factor / Q000378:metabolism
D000941:Antigens; D037102:Lectins; D037121:Plant Lectins; C036850:Ulex europaeus lectins; D014746:Vimentin; D000509:alpha-Fetoproteins; D014841:von Willebrand Factor; D014642:Vanilmandelic Acid; D005169:Factor VIII; D006719:Homovanillic Acid