Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging.

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.

[Spontaneous regression of familial glioma of the optic nerve in a boy with suspected neurofibromatosis 1 (Recklinghausen's disease)]. / Spontannaia regressiia semeinoi gliomy zritel'nogo nerva u mal'chika s predpolagaemym neirofibromatozom I tipa (bolezn' Reklingauzena). .

A boy with a large intracranial glioma of the optic tract and probable neurofibromatosis of the first type was observed for 8 years since the age of 7 years. A series of MR scans was made over this period. A notable decrease of the tumor size was seen on its signals on the MR scans. This was paralleled by an improvement of the vision acuity, color field, and visual field on the involved eye. Patient's grandmother had an intracranial glioma of the optic nerve with a slight but stable decrease of the visual functions. The tumor shape in the grandmother and grandchild is remarkably similar. This finding in the grandmother and stability of her vision decreased from childhood permit us to propose that the tumor did not develop and even regressed with time.