Intraorbital and intracranial soft-tissue glomus tumor in an 8-year-old child. Case report

J Neurosurg Pediatr. 2008 May;1(5):389-91. doi: 10.3171/PED/2008/1/5/389.

Abstract

Soft-tissue glomus tumors (or glomangiomas) are unrelated to neuroendocrine paragangliomas (glomus tympanicum, jugulare, and vagale). The authors present the first reported case of an orbital soft-tissue glomus tumor in a child. An 8-year-old girl developed rapidly progressive right-eye blindness, proptosis, and a sixth cranial nerve palsy. Magnetic resonance imaging demonstrated a homogeneously enhancing lesion extending from the right orbit through the superior orbital fissure to the cavernous sinus and middle cranial fossa. A biopsy specimen demonstrated the lesion to be a soft-tissue glomus tumor. Following angiography and embolization, a gross-total resection of the tumor was achieved. The patient was treated with adjuvant proton-beam radiotherapy. At 24 months follow-up her proptosis and sixth cranial nerve palsy had resolved and there was no evidence of tumor recurrence.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Diagnosis, Differential
  • Female
  • Glomus Tumor / pathology*
  • Glomus Tumor / therapy
  • Humans
  • Orbital Neoplasms / pathology*
  • Orbital Neoplasms / therapy
  • Paraganglioma / diagnosis