Spontaneous Partial Regression of Low-Grade Glioma in Children With Neurofibromatosis-1: A Real Possibility

Abstract

At the age of 41 and 31 months, respectively, a boy and a girl affected by neurofibromatosis-1 were diagnosed with a visual pathway glioma during surveillance contrast-enhanced head magnetic resonance imaging (MRI). In the first child, the initial MRI showed that the entire optic chiasm, the intracranial tract of the left optic nerve, and hypothalamus were grossly enlarged and enhanced in the post-gadolinium T1-weighted images. Ten months later, the hypothalamic component of the lesion had regressed markedly and there were no more areas of contrast enhancement. In the second child, the initial MRI showed that the optic chiasm, the right optic tract, and geniculate body were enlarged and enhanced after gadolinium injection. At 6-month follow-up, the MRI showed that the right optic tract and the anterior aspect of the optic chiasm decreased in size and the contrast enhancement of the entire lesion was reduced dramatically. These findings, as indicated by other similar reports, confirm that spontaneous regression of visual pathway glioma is a rare but real possibility in children with neurofibromatosis-1. Therefore, clinicians need to be aware of visual pathway glioma's erratic behavior in children with neurofibromatosis-1 with special attention given to the importance of a very conservative attitude toward any type of treatment for such patients.

Optic pathway gliomas: a review

“…Although authors of numerous case reports have described spontaneous regression of OPG after surgical debulking or biopsy without additional postoperative adjuvant therapy, 25,28,54,55,84,85 it is unclear whether spontaneous regression might have occurred without any treatment at all, which has also been well-reported. 63,66 In 2006, Ahn et al 2 retrospectively reviewed a series of 33 patients with OPGs who had undergone surgery over a 17-year period. They found that radical removal of OPGs was of no survival benefit and that it did not reduce endocrine complications.…”

“…Another argument for observing patients with newly diagnosed OPGs is the fact that spontaneous regression without treatment has been well documented in patients with and those without NF1. 25,48,49,63,65,66 Neurosurg. Focus In a major review of 1136 cases for which treatment data were available, Dutton 15 found that 40% of patients with OPGs of the anterior visual pathway experienced progression or recurrence.…”

Spontaneous Regression of Low-Grade Gliomas in Pediatric Patients without Neurofibromatosis

“…Of the factors that have been suggested as positive associations for tumor regression, the association with neurofibromatosis has been the more widely reported. Over 15 reports of the spontaneous regression of low-grade gliomas in association with neurofibromatosis type 1 have been reported [5,6,7,8,9,10,11,12]. The consideration of spontaneous regression is significant, as it would allow a significant number of patients to avoid the side effects of treatment.…”

Improvement of Visual Acuity Caused by Opticochiasmal Astrocytoma After Treatment for Associated Hydrocephalus-Case Report-

“…3,12,15,19,23) Spontaneous regression without surgical intervention has been also reported, but the diagnosis was based on radiological findings. 2,8,12,13,15,16,21) Regardless of the method used for diagnosis, subgroups of this entity are clearly self-limiting and can demonstrate spontaneous regression. Several mechanisms underlying the regression or growth arrest of low-grade glioma have been suggested, including the activation of host immunity after surgical intervention, apoptosis or programmed cell death, and endocrine-assisted reduction in vascular engorgement of the tumor.…”