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Spontaneous regression of optic pathways gliomas in three patients with neurofibromatosis type I and critical review of the literature.

CASE REPORTS: The authors report their experience about three children (two girls, one boy; average age 1.6 years) with a spontaneous regression of optic gliomas. All of them had a previous diagnosis of neurofibromatosis type 1 (NF 1). None of them underwent surgery or biopsy nor received chemotherapy or radiotherapy. The complete regression was documented by MRI scans performed during a mean follow-up of 6.3 years. LITERATURE REVIEW: Moreover, the authors analyze the features of the 16 cases previously reported in English literature of spontaneously regressed optic gliomas with an overview of the different therapeutic strategies. The knowledge that this kind of tumor, particularly in young patients, may regress is important in the decision of the best therapeutic approach.

Child, Preschool↗

The biological and clinical behaviour of pilocytic astrocytomas of the optic pathways.

The clinical and pathological findings in thirty patients with pilocytic astrocytomas of the anterior visual pathways were analysed. Our cases and those in the literature did not show positive correlation between the extent of surgical resection or irradiation or the presence of residual tumour, and the length of survival or degree of residual disability. Most of these tumours may be considered hamartoma-like growths, akin to developmental malformations, among which aggressive behaviour is exceptional. Their inherently benign nature, associated with long periods of dormancy and spontaneous regression, indicate that treatment should be conservative, and that a surprisingly favourable outcome can often be anticipated.

Adolescent↗

Evidence of optic pathway gliomas after previously negative neuroimaging.

PURPOSE: The authors emphasize the potential for the development of anterior visual pathway gliomas, evidenced by computed tomography (CT) or magnetic resonance imaging (MRI) scans, in neurofibromatosis type 1 (NF1) patients who previously had normal neuroimaging studies. METHODS: The clinic charts and CT and MRI scans were retrospectively reviewed for all patients evaluated at the neurofibromatosis clinic of one referral center over a period of 7 years. Patients with neuroimaging studies demonstrating anterior visual pathway gliomas who previously had normal scans were identified, and their cases are described in detail. A similar, previously reported series, from the pediatric literature, was also reviewed. RESULTS: Eight percent (28/360) of patients had CT or MRI scans revealing optic gliomas. Two of these patients had normal neuroimaging studies previously. CONCLUSION: A negative neuroimaging study in an NF1 patient does not exclude the future development of an optic glioma.

Antineoplastic Combined Chemotherapy Protocols↗

Disruption of the optic pathway during development affects vasoactive intestinal peptide mRNA expression.

Vasoactive intestinal polypeptide (VIP) is a regulatory peptide widely distributed in the central and peripheral nervous systems. To understand the activities of VIP it is necessary to study the mechanisms governing its production. The highest concentration of VIP-producing cells occurs in the suprachiasmatic nucleus (SCN) of the hypothalamus. Because the SCN is directly innervated by the optic nerve, we decided to investigate the effect of visual input on VIP gene expression. By means of Northern blot hybridization, we measured VIP mRNA levels in the hypothalami of 36-day-old normal rats and rats that had been enucleated at birth. The concentration of VIP mRNA in the hypothalami of enucleated rats was approximately double that in the hypothalami of normal rats. In contrast, the concentration of VIP mRNA in the cerebral cortex significantly decreased after enucleation. The concentrations of VIP mRNA were also measured by in situ hybridization to brain sections. The hypothalamic VIP mRNA was located mainly in the SCN. Enucleation resulted in an increase of VIP transcripts in the SCN. These results indicate that visual input may participate in the regulation of VIP production.

Animals↗