Primary capillary hemangioblastoma of peripheral soft tissues

Am J Surg Pathol. 2004 Jul;28(7):962-6. doi: 10.1097/00000478-200407000-00018.

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.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Ankle
  • Antigens, CD34 / analysis
  • Calcium-Binding Proteins
  • Calponins
  • Endothelium, Vascular / chemistry
  • Female
  • Hemangioblastoma / chemistry
  • Hemangioblastoma / pathology*
  • Hemangioblastoma / ultrastructure
  • Humans
  • Immunohistochemistry
  • Microfilament Proteins
  • Phosphopyruvate Hydratase / analysis
  • Platelet Endothelial Cell Adhesion Molecule-1 / analysis
  • S100 Proteins / analysis
  • Soft Tissue Neoplasms / chemistry
  • Soft Tissue Neoplasms / pathology*
  • Soft Tissue Neoplasms / ultrastructure
  • Vimentin / analysis
  • von Willebrand Factor / analysis

Substances

  • Antigens, CD34
  • Calcium-Binding Proteins
  • Microfilament Proteins
  • Platelet Endothelial Cell Adhesion Molecule-1
  • S100 Proteins
  • Vimentin
  • von Willebrand Factor
  • Phosphopyruvate Hydratase