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September 1971

Benign Orbital Neurilemoma: Sarcomatous Transformation in von Recklinghausen's Disease

Author Affiliations

MacDill Air Force Base, Fla
From the Illinois Eye and Ear Infirmary, Chicago. Dr. Schatz is now with the Wilmer Eye Institute, Baltimore.

Arch Ophthalmol. 1971;86(3):268-273. doi:10.1001/archopht.1971.01000010270006

NEURILEMOMAS (benign schwannomas) are found in 1.5%1 to 18%2,3 of patients with von Recklinghausen's neurofibromatosis and make up 1% of orbital tumors.4-9 They rarely, if ever, undergo malignant change.2,10-17

This paper describes, to this author's knowledge, the first reported case of sarcomatous transformation of a benign neurilemoma of the orbit.

Report of a Case  A 27-year-old white woman (51-38-29) was first seen at the University of Illinois Eye Clinic on Nov 21, 1966, and was admitted to the hospital with a six-week history of a mass in the superior right orbit. Two weeks previous to admission she noted gradual proptosis and some pain. Additional history revealed the mother and sister had café-au-lait spots. Examination of the patient showed a right superior orbital mass with some proptosis and mechanical limitation of upward gaze. The mass was firm, discrete, round, and movable. Vision was 20/40 OD, 20/30 OS.

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