title: Primary capillary hemangioblastoma of peripheral soft tissues.
abstract: A case of capillary hemangioblastoma located in the peripheral soft tissue of the inner ankle in a 74-year-old woman is presented. The tumor was an unencapsulated but sharply circumscribed nodule 2.5 cm in size, of a yellow-white color. It showed reddish-brown spots with small cysts up to 2 mm filled with blood. Grossly the tumor was not attached to any peripheral nerve. Signs of von Hippel-Lindau's disease were excluded by thorough clinical evaluation. No additional tumor or erythrocytosis was found in the patient clinically. Immunohistochemically, the tumor stromal cell reacted strongly with antibodies to S-100 protein, NSE, and calponin and they were negative with antibodies to GFAP, CD34, CD31, cytokeratins, actin, desmin, EMA, and HMB-45. Endothelium of the capillaries reacted positively with antibodies to CD31, CD34, and Factor VIII-related protein. Capillary pericytes were actin-positive. All cells of the tumor stained positively with antibody to vimentin. MIB1 antibody reacted only in very few cells (<1%). Ultrastructurally, the stromal cells contained electron-lucent cytoplasm with lipid droplets, a small amount of rough endoplasmic reticulum, and glycogen particles. No electron-dense structures typical of secretory granules were seen in the stromal cells. No mutation of coding sequence of VHL gene was found.
authors.affiliation: Department of Pathology, Faculty Hospital, Pilsen, Czech Republic. michal@medima.cz
authors.forename: Michal
authors.initials: M
authors.lastname: Michal
authors.forename: Tomas
authors.initials: T
authors.lastname: Vanecek
authors.forename: Radek
authors.initials: R
authors.lastname: Sima
authors.forename: Petr
authors.initials: P
authors.lastname: Mukensnabl
authors.forename: Ludmila
authors.initials: L
authors.lastname: Boudova
authors.forename: Martina
authors.initials: M
authors.lastname: Brouckova
authors.forename: Karep
authors.initials: K
authors.lastname: Koudepa
mesh_terms: D000368:Aged; D000842:Ankle; D018952:Antigens, CD34 / Q000032:analysis; D002135:Calcium-Binding Proteins; D004730:Endothelium, Vascular / Q000737:chemistry; D005260:Female; D018325:Hemangioblastoma / Q000737:chemistry / Q000473:pathology* / Q000648:ultrastructure; D006801:Humans; D007150:Immunohistochemistry; D008840:Microfilament Proteins; D010751:Phosphopyruvate Hydratase / Q000032:analysis; D019408:Platelet Endothelial Cell Adhesion Molecule-1 / Q000032:analysis; D009418:S100 Proteins / Q000032:analysis; D012983:Soft Tissue Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000648:ultrastructure; D014746:Vimentin / Q000032:analysis; D014841:von Willebrand Factor / Q000032:analysis; D000096985:Calponins
chemical_list: D018952:Antigens, CD34; D002135:Calcium-Binding Proteins; D008840:Microfilament Proteins; D010751:Phosphopyruvate Hydratase; D019408:Platelet Endothelial Cell Adhesion Molecule-1; D009418:S100 Proteins; D014746:Vimentin; D014841:von Willebrand Factor; D000096985:Calponins