Soft-tissue hemangioblastoma of the retroperitoneum: a case study and review of the literature

Appl Immunohistochem Mol Morphol. 2010 Oct;18(5):479-82. doi: 10.1097/PAI.0b013e3181daad26.


A 71-year-old woman was found to have a solid retroperitoneal mass measuring 7 x 6 cm on a computed tomography scan. The tumor was not connected to the spinal cord or large nerves. It was resected and showed the typical histology of hemangioblastoma, that is, numerous capillaries and stromal cells with focal cytoplasmic vacuolization. Immunohistochemical study revealed that the tumor stromal cells were positive for S-100 protein, neuron-specific enolase, and inhibin-α, supporting the diagnosis. In addition, the tumor cells labeled for carbonic anhydrase IX and brachyury. The diffuse strong carbonic anhydrase IX expression suggested that soft-tissue hemangioblastoma may share with central nervous system hemangioblastoma the tumorigenic mechanism involving the von Hippel-Lindau gene product and hypoxia-inducible factor. The brachyury expression observed in this case, albeit focal and weak, may implicate embryonic hemangioblasts as a potential line of differentiation, as was proposed in central nervous system hemangioblastoma. The patient is well 4 years after the surgery without any recurrence. A review of the literature revealed that soft-tissue hemangioblastoma is typically a solid tumor found in older females and often involves the retroperitoneum/pelvis.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Disease-Free Survival
  • Female
  • Fetal Proteins / metabolism
  • Hemangioblastoma / diagnosis*
  • Hemangioblastoma / metabolism
  • Hemangioblastoma / pathology
  • Hemangioblastoma / surgery
  • Humans
  • Immunohistochemistry
  • Inhibins / metabolism
  • Phosphopyruvate Hydratase / metabolism
  • Retroperitoneal Neoplasms / diagnosis*
  • Retroperitoneal Neoplasms / metabolism
  • Retroperitoneal Neoplasms / pathology
  • Retroperitoneal Neoplasms / surgery
  • S100 Proteins / metabolism
  • Soft Tissue Neoplasms / diagnosis*
  • Soft Tissue Neoplasms / metabolism
  • Soft Tissue Neoplasms / pathology
  • Soft Tissue Neoplasms / surgery
  • T-Box Domain Proteins / metabolism
  • Tomography, X-Ray Computed
  • Von Hippel-Lindau Tumor Suppressor Protein / metabolism*


  • Fetal Proteins
  • S100 Proteins
  • T-Box Domain Proteins
  • inhibin-alpha subunit
  • Inhibins
  • Von Hippel-Lindau Tumor Suppressor Protein
  • Phosphopyruvate Hydratase
  • Brachyury protein