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

Hematology-Oncology Division, Padua University Hospital, Pediatric Neuro-Oncology Program, Padua, Italy

Paola Moras, MD

Neurology Division, Padua University Hospital, Pedicatric Neuro-Oncology Program, Padua, Italy

Carla Carollo, MD

Department of Pediatrics, the Division of Neuroradiology, Padua University Hospital, Pediatric Neuro-Oncology Program, Padua, Italy

Antonio Battistella, MD

Neurology Division, Padua University Hospital, Pedicatric Neuro-Oncology Program, Padua, Italy

Maurizio Clementi, MD

Divison of Medical Genetics Padua University Hospital, Pediatric Neuro-Oncology Program, Padua, Italy

AnnaMaria Laverda, MD

Neurology Division, Padua University Hospital, Pedicatric Neuro-Oncology Program, Padua, Italy

Alessandra Murgia, PhD, MD

Molecular Biology Unit, Padua University Hospital, Pedicatric Neuro-Oncology Program, Padua, Italy

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. (J Child Neurol 1999;14:352-356).

Journal of Child Neurology, Vol. 14, No. 6, 352-356 (1999)
DOI: 10.1177/088307389901400602


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