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.
Schatz H. Benign Orbital Neurilemoma: Sarcomatous Transformation in von Recklinghausen's Disease. Arch Ophthalmol. 1971;86(3):268–273. doi:10.1001/archopht.1971.01000010270006
Coronavirus Resource Center
Customize your JAMA Network experience by selecting one or more topics from the list below.