Giant cell angiofibroma, a variant of solitary fibrous tumor, of the orbit in a 16-year-old girl

Ophthalmic Plast Reconstr Surg. 2009 Sep-Oct;25(5):402-4. doi: 10.1097/IOP.0b013e3181b39a15.


A 16-year-old girl presented with diplopia and gradual-onset, painless proptosis of the left eye. Orbital CT showed a well-circumscribed, enhancing, extraconal mass in the superior orbit, and the surgical excision was performed. Histopathology was interpreted as capillary hemangioma. Five years later, her symptoms recurred, and she was referred to the Oncology Service, Wills Eye Institute. Repeat orbital MRI showed a well-defined, extraconal mass with loculated areas of enhancement in the left orbit superonasally. Complete surgical excision was performed. Histopathologic examination showed benign, patternless spindle-cell proliferation with prominent intrinsic vascularity and multinucleated giant cells, consistent with giant cell angiofibroma, a variant of solitary fibrous tumor. There was intense immunoreactivity for CD34. After 20 months follow-up, there was no recurrence or development of metastasis. Giant cell angiofibroma, a variant of solitary fibrous tumor, is a rare orbital tumor that presents as a well-circumscribed, enhancing mass and can be found in children.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Angiofibroma / metabolism
  • Angiofibroma / pathology*
  • Angiofibroma / surgery
  • Antigens, CD34 / metabolism
  • Biomarkers, Tumor / metabolism
  • Diplopia / diagnosis
  • Exophthalmos / diagnosis
  • Female
  • Giant Cell Tumors / metabolism
  • Giant Cell Tumors / pathology*
  • Giant Cell Tumors / surgery
  • Humans
  • Magnetic Resonance Imaging
  • Ophthalmologic Surgical Procedures
  • Orbital Neoplasms / metabolism
  • Orbital Neoplasms / pathology*
  • Orbital Neoplasms / surgery
  • Solitary Fibrous Tumors / metabolism
  • Solitary Fibrous Tumors / pathology*
  • Solitary Fibrous Tumors / surgery


  • Antigens, CD34
  • Biomarkers, Tumor