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January 1990

Multiple Orbital Neurofibromas Unassociated With von Recklinghausen's Disease

Author Affiliations

From the Ocular Oncology Service (Drs J. Shields, C. Shields, and Lieb) and the Pathology Department (Dr Eagle), Wills Eye Hospital, Jefferson Medical College, Thomas Jefferson University, Philadelphia, Pa.

Arch Ophthalmol. 1990;108(1):80-83. doi:10.1001/archopht.1990.01070030086034

• A 58-year-old man with a 10-year history of right periorbital pain treated with multiple analgesics presented with slowly progressive exophthalmos of the right eye. Orbital imaging studies disclosed three separate well-defined tumors, located in the temporal fossa, the intraconal space, and within the floor of the orbit. All three tumors were removed intact and proved on histopathologic evaluation to be localized neurofibromas. The patient had complete relief of the chronic pain following removal of the tumors. We emphasize that the unusual occurrence of multiple circumscribed orbital tumors should suggest the diagnosis of neurofibroma even in the absence of von Recklinghausen's neurofibromatosis.