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Spontaneous Regression of Optic Gliomas
Thirteen Cases Documented by Serial Neuroimaging
Cameron F. Parsa, MD;
Creig S. Hoyt, MD;
Robert L. Lesser, MD;
Joel M. Weinstein, MD;
Charles M. Strother, MD;
Rafael Muci-Mendoza, MD;
Marcos Ramella, MD;
Riri S. Manor, MD;
William A. Fletcher, MD;
Michael X. Repka, MD;
James A. Garrity, MD;
Roberto N. Ebner, MD;
Mario L. R. Monteiro, MD;
Robert M. McFadzean, MD;
Irina V. Rubtsova, MD;
William F. Hoyt, MD
Arch Ophthalmol. 2001;119:516-529.
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.
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).
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