PubMed · 4460091
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K R Hill, R W Joyner. 1974. The EMI-scanner.. https://pubmed.ncbi.nlm.nih.gov/4460091/
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OBJECTIVE: To distinguish the clinical manifestations and natural history of optic pathway tumors (OPT) associated with neurofibromatosis type 1 (NF-1 OPT) from that of OPT not associated with NF-1 (non-NF-1 OPT). METHODS: Two groups of children with OPT were compared: (1) 17 children with NF-1 OPT who were followed prospectively, and (2) 19 children with non-NF-1 OPT who were identified retrospectively by a review of medical records. RESULTS: Precocious puberty was a common initial sign in the children with NF-1 OPT (5/17), and was not found in any patients without NF-1. In contrast, children with non-NF-1 OPT had symptoms attributable to increased intracranial pressure (12/19 and nystagmus (5/19); these symptoms were not found in any patient with NF-1. Decreased visual acuity at the time of diagnosis was common in both groups. There was no significant difference between the children with NF-1 OPT and those with non-NF-1 OPT as to age at diagnosis or sex distribution. Optic nerve involvement was more common in NF-1 (p < 0.001). Both isolated and bilateral optic nerve tumors were found exclusively in children with NF-1, whereas chiasmal (p = 0.016) and optic tract involvement (p = 0.001) were more common in those with non-NF-1 OPT. Radiographic evidence of hydrocephalus was found in none of the children with NF-1 OPT compared with 79% of the non-NF-1 OPT group. Progressive disease was seen in 12% of patients with NF-1 OPT compared with 63% of those with non-NF-1 OPT. CONCLUSIONS: Differences exist between NF-1 OPT and non-NF-1 OPT both at the time of diagnosis and during follow-up. Optic pathway tumors caused by NF-1 and non-NF-1 OPT have different biologic properties that distinguish both their initial clinical manifestations and their natural history.
Malignant gliomas are the most common primary human brain tumours, but their classification remains controversial and effective therapies remain elusive. As a result, malignant gliomas have come under intense scientific scrutiny, in the hope of elucidating the molecular basis of glial tumourigenesis. These studies have yielded insights into the genetic events that underlie glioma formation and progression, and have shown multiple distinct genetic pathways that lead to the common malignant endpoint of glioblastoma multiforme. Such genetic pathways mirror clinicopathological avenues of glioma progression and suggest that molecular genetic approaches might have clinical utility in the coming years.
We report a fatal tumor hemorrhage in a 1-and-a-half-year old girl after ventriculoperitoneal shunting for obstructive hydrocephalus caused by a thalamic tumor. The possible pathophysiological mechanism is briefly discussed and the literature is reviewed.