Primary capillary hemangioblastoma of peripheral soft tissues.
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.
Michal
Michal
M
Department of Pathology, Faculty Hospital, Pilsen, Czech Republic. michal@medima.cz
Vanecek
Tomas
T
Sima
Radek
R
Mukensnabl
Petr
P
Boudova
Ludmila
L
Brouckova
Martina
M
Koudepa
Karep
K
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
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