Spontaneous regression of optic gliomas - Thirteen cases documented by serial neuroimaging

Objective: To demonstrate spontaneous regression of large, clinically sympt omatic optic pathway gliomas in patients with and without neurofibromatosis type I (NF-I). Methods: Patient cases were collected through surveys at 2 consecutive annu al meetings of the North American Neuro-Ophthalmology Society (NANOS) and t hrough requests on the NANOSNET Internet listserv. Serial documentation of tumor signal and size, using magnetic resonance imaging in II patients and computed tomography in 2 patients, was used to evaluate clinically symptoma tic optic pathway gliomas. All tumors met radiologic criteria for the diagn osis of glioma and 4 patients had biopsy confirmation of their tumors. In 3 patients, some attempt at therapy had been made many years before regressi on occurred. In one of these, radiation treatment had been given 19 years b efore tumor regression, while in another, chemotherapy had been administere d 5 years before signal changes in the tumor. In the third patient, minimal surgical debulking was performed I year before the tumor began to shrink. Results: Spontaneous tumor shrinkage was noted in 12 patients. Eight patien ts did not have NF-I. In an additional patient without NF-1, a signal chang e within the tumor without associated shrinkage was detected. Tumor regress ion 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 sponta neous regression. Regression was seen in patients with and without NF-1. Re gression may manifest either as an overall shrinkage in tumor size, or as a signal change on magnetic resonance imaging. A variable degree of improvem ent in visual function may accompany regression. The possibility of spontan eous regression of an optic glioma should be considered in the planning of treatment of patients with these tumors.