Multiple orbital neurofibromas unassociated with von Recklinghausen's disease
J. A. Shields, C. L. Shields, W. E. Lieb and R. C. Eagle
Ocular Oncology Service, Wills Eye Hospital, Jefferson Medical College, Thomas Jefferson University, Philadelphia, Pa 19107.
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.
