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Clinical Sciences
April 2001

Spontaneous Regression of Optic Gliomas: Thirteen Cases Documented by Serial Neuroimaging

Author Affiliations

From the Wilmer Ophthalmological Institute, Johns Hopkins University School of Medicine, Baltimore, Md (Drs Parsa and Repka); Department of Ophthalmology, San Francisco School of Medicine, University of California, San Francisco(Drs C. Hoyt and W. Hoyt); the Department of Ophthalmology and Visual Science, Yale University School of Medicine, New Haven, Conn (Dr Lesser); the Departments of Ophthalmology and Visual Sciences (Dr Weinstein) and Radiology (Dr Strother), University of Wisconsin School of Medicine, Madison; Neuro-ophthalmology Unit, Hospital Vargas, Caracas, Venezuela (Drs Muci-Mendoza and Ramella); Neuro-ophthalmology Unit, Department of Ophthalmology, Sapir Medical Center, Meir Hospital, Kfar-Saba, Israel (Dr Manor); the Departments of Clinical Neurosciences and Surgery (Ophthalmology), University of Calgary, Calgary, Alberta (Dr Fletcher); Department of Ophthalmology, Mayo Clinic, Rochester, Minn (Dr Garrity); Neuro-ophthalmology Unit, British Hospital, Buenos Aires, Argentina (Dr Monteiro); Department of Ophthalmology, Faculty of Medicine of the University of São Paulo, São Paulo, Brazil (Dr Monteiro); Neuro-ophthalmology Unit, Institute of Neurological Sciences, Southern General Hospital, Glasgow, Scotland (Dr McFadzean); and the Department of Ophthalmology, Pavlov State Medical University, St Petersburg, Russia (Dr Rubtsova).

Arch Ophthalmol. 2001;119(4):516-529. doi:10.1001/archopht.119.4.516
Abstract

Objective  To demonstrate spontaneous regression of large, clinically symptomatic optic pathway gliomas in patients with and without neurofibromatosis type 1 (NF-1).

Methods  Patient cases were collected through surveys at 2 consecutive annual meetings of the North American Neuro-Ophthalmology Society (NANOS) and through requests on the NANOSNET Internet listserv. Serial documentation of tumor signal and size, using magnetic resonance imaging in 11 patients and computed tomography in 2 patients, was used to evaluate clinically symptomatic optic pathway gliomas. All tumors met radiologic criteria for the diagnosis of glioma and 4 patients had biopsy confirmation of their tumors. In 3 patients, some attempt at therapy had been made many years before regression occurred. In one of these, radiation treatment had been given 19 years before tumor regression, while in another, chemotherapy had been administered 5 years before signal changes in the tumor. In the third patient, minimal surgical debulking was performed 1 year before the tumor began to shrink.

Results  Spontaneous tumor shrinkage was noted in 12 patients. Eight patients did not have NF-1. In an additional patient without NF-1, a signal change within the tumor without associated shrinkage was detected. Tumor regression was associated with improvement in visual function in 10 of 13 patients, stability of function in 1, and deterioration in 2.

Conclusions  Large, clinically symptomatic optic gliomas may undergo spontaneous regression. Regression was seen in patients with and without NF-1. Regression may manifest either as an overall shrinkage in tumor size, or as a signal change on magnetic resonance imaging. A variable degree of improvement in visual function may accompany regression. The possibility of spontaneous regression of an optic glioma should be considered in the planning of treatment of patients with these tumors.

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