title: Primary hepatic epithelioid hemangioendothelioma progressively responsive to interferon-alpha: is there room for novel anti-angiogenetic treatments?
abstract: Primary hepatic epithelioid hemangioendothelioma (HEH) is a rare, low-grade malignant neoplasm of endothelial origin, with an unpredictable clinical course and prognosis. No standard therapeutic strategies are still available for HEH, due to the infrequency of the disease and to its variable natural history that limit the identification of the most effective treatment. In the absence of metastatic disease, surgical resection or liver transplantation represent the treatment of choice for HEH, while several antineoplastic agents have been proposed in the presence of metastatic nonresectable disesase. Herein, we describe the biological characterization and the clinical course of a primary HEH progressively responsive to treatment with intermediate doses of interferon-alpha (IFN)-alpha2a. Furthermore, based on the newly-identified expression of endoglin (CD105) on HEH, we discuss the clinical potential of novel anti-angiogenetic approaches to the disease.
authors.affiliation: Division of Medical Oncology and Immunotherapy, Department of Oncology University Hospital of Siena, Siena, Italy.
authors.forename: L
authors.initials: L
authors.lastname: Calabrò
authors.forename: A M
authors.initials: AM
authors.lastname: Di Giacomo
authors.forename: M
authors.initials: M
authors.lastname: Altomonte
authors.forename: E
authors.initials: E
authors.lastname: Fonsatti
authors.forename: M A
authors.initials: MA
authors.lastname: Mazzei
authors.forename: L
authors.initials: L
authors.lastname: Volterrani
authors.forename: C
authors.initials: C
authors.lastname: Miracco
authors.forename: M
authors.initials: M
authors.lastname: Maio
mesh_terms: D020533:Angiogenesis Inhibitors / Q000627:therapeutic use*; D015703:Antigens, CD / Q000032:analysis; D018952:Antigens, CD34 / Q000032:analysis; D000071063:Endoglin; D005260:Female; D018323:Hemangioendothelioma, Epithelioid / Q000098:blood supply / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use*; D008113:Liver Neoplasms / Q000098:blood supply / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D008875:Middle Aged; D009389:Neovascularization, Pathologic / Q000276:immunology / Q000473:pathology / Q000517:prevention & control*; D019408:Platelet Endothelial Cell Adhesion Molecule-1 / Q000032:analysis; D011956:Receptors, Cell Surface / Q000032:analysis; D011994:Recombinant Proteins; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D020533:Angiogenesis Inhibitors; D015703:Antigens, CD; D018952:Antigens, CD34; C064206:ENG protein, human; D000071063:Endoglin; D000077190:Interferon alpha-2; D016898:Interferon-alpha; D019408:Platelet Endothelial Cell Adhesion Molecule-1; D011956:Receptors, Cell Surface; D011994:Recombinant Proteins