Intraorbital and intracranial soft-tissue glomus tumor in an 8-year-old child
Case report Todd C. Hankinson, M.D.1, Alfred T. Ogden, M.D.1, Peter Canoll, M.D., Ph.D.2, James H. Garvin, M.D.3, Michael Kazim, M.D.4, Jeffrey N. Bruce, M.D.1, Neil A. Feldstein, M.D.1, and Richard C. E. Anderson, M.D.1 1Departments of Neurosurgery; 2Pathology, Division of Neuropathology; 3Pediatrics, Division of Hematology/Oncology; and 4Ophthalmology and Surgery, Columbia University, College of Physicians and Surgeons, New York, New York Abbreviation used in this paper: MR = magnetic resonance. Address correspondence to: Todd C. Hankinson, M.D., Department of Neurosurgery, Columbia University, College of Physicians and Surgeons, 710 West 168th Street, NI Box #132, New York, New York 10032. email: tch12@columbia.edu. DOI: 10.3171/PED/2008/1/5/389 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.
KEYWORDS:glomangioma; glomus body; glomus tumor; paraganglioma.
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