Optic Nerve Glioma and Cerebellar Astrocytoma in a Patient With Von Recklinghausen'S Neurofibromatosis

Miller, Neil R.

doi:10.1016/0002-9394(75)90796-5

Cited by 10 publications

(4 citation statements)

References 8 publications

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“…In our experience, patients with CGNF1 were older than those with NF1-associated optic gliomas, which could suggest that the glial migration and maturation patterns under the dependence of the neurofibromin are different in these two sites. In addition, two of our patients had an optic tract glioma several years before the occurrence of CGNF1, a finding reported earlier [5], which could suggest the existence of a subset of NF1 patients with greater than normal vulnerability for glial tumors. Future genetic studies could yield molecular support for these hypotheses.…”

Section: Discussionsupporting

confidence: 48%

Cerebellar gliomas in children with NF1: pathology and surgery

Vinchon¹,

Soto-Ares

et al. 2000

Child's Nerv Syst

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Cerebellar gliomas associated with NF1 (CGNF1) are rarely reported in the literature, and they are considered to be malignant in a high proportion of cases. In an attempt to improve the definition of this disease and clarify its management, we reviewed our patients with CGNF1 and compared their tumors with sporadic cerebellar gliomas (SGC). We operated on six children with CGNF1, all but one of whom were asymptomatic. They represented one-tenth of all pediatric cerebellar gliomas, and one third of NF1-associated gliomas seen in our institution. CGNF1 appeared at a later age than SCG. They are seated near the roof of the IV ventricle and are not related to white matter hypersignal hamartomas. Most of these tumors showed radiological progression. They were four pilocytic astrocytomas, one ganglioglioma, and one malignant astrocytoma. One patient had tumor recurrence after 8 years, and the others are still disease free. The overall outcome appeared to be better for GCNF1 than for SCG. On account of the regular growth, uncertain pathology, and good surgical outcome, we advocate systematic resection of these tumors.

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Section: Discussionsupporting

confidence: 48%

Cerebellar gliomas in children with NF1: pathology and surgery

Vinchon¹,

Soto-Ares

et al. 2000

Child's Nerv Syst

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“…The strong bright signals produced by the lesions on T2W images, the so‐called ‘unidentified bright objects’ (UBOs) have recently been reported to indicate hamartomas of the cerebellum, brainstem, basal ganglia, thalamus and hippocampus. Cerebellar hamartoma, an unusual finding of the case, has only been reported in chidren . Thus, the presence of UBOs is yet another criterion for NF1.…”

Section: Reportmentioning

confidence: 74%

“…Cerebellar hamartoma, an unusual finding of the case, has only been reported in chidren. 8,9 Thus, the presence of UBOs 10 is yet another criterion for NF1. Vacuolar changes in the myelin, such as spongiform myelinopathy, may lead to upper signal intensity of lesions on T2W images.…”

Section: Reportmentioning

confidence: 99%

Plexiform neurofibroma affecting the upper parietal scalp, with cerebellar hamartoma: role of histopathology, colour Doppler imaging and magnetic resonance imaging

Sehgal¹,

Oberai²,

Venkatash

et al. 2013

Clinical and Experimental Dermatology

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We report a patient with plexiform neurofibroma, which is pathognomonic for neurofibromatosis type 1 (NF1) affecting the upper parietal region of the scalp. Cerebellar hamartoma was present, a finding that, to our knowledge, has not been reported previously. We highlight the role of histopathology, ultrasonography, colour Doppler imaging and magnetic resonance imaging, in addition to the seven existing criteria, for the diagnosis of NF1.

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“…The frequency of association of neurofibromatosis and optic nerve glioma may be underestimated due to the usual development of cafe-au-lait spots at puberty or later, whereas the highest incidence of glioma is in the first decade. Patients with neurofibromatosis may have multiple intracranial tumors (Rodriguez & Berthong, 1966); however, only one case in which an optic nerve glioma was associated with another primary intracranial tumor has been reported (Miller, 1975).…”

Section: Clinical Featuresmentioning

confidence: 99%

Tumors of the optic nerve

1976

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This is a review of primary and secondary tumors of the optic nerve. The emphasis is an optic nerve gliomas and meningiomas. Optic nerve gliomas are slowly growing astrocytic neoplasms of the anterior visual pathways, the majority of which occur within the first two decades of life, with equal sex incidence in about 1 of 200,000 patients presenting with eye complaints. The incidence is greater in neurofibromatosis. The typical presentation is visual impairment in a verbal pre-school child with optic canal enlargement and optic atrophy. An intraorbital location of the tumor leads to axial, irreducible, non-pulsatile proptosis. An intracranial location may disturb hypothalamic and pituitary function and produce hydrocephalus. Ocular findings may also include limited motility on a mechanical-restrictive basis, a pupillary relative afferent defect, nystagmus, and variable, non-specific visual field defects. Roentgenographic studies may show concentric unilateral enlargement of the optic canal with preservation of a well corticated margin, a fossa under the anterior clinoid process in continuity with the optic canal ('J'-shaped sella), and findings of increased intracranial pressure. On pathologic examination the tumor is a smooth, fusiform, intradural enlargement of the optic nerve. Histologically there is proliferation of elongated (pilocytic) astrocytes in reticulated patterns with intervening microcystic spaces containing mucosubstance and surrounding reactive hyperplasia of the arachnoid. Mitoses are not found. The diagnosis is clinical X-ray studies and brain scan should be performed. The differential diagnosis is that of unilateral proptosis in a child and includes acute ethmoiditis, hyperthyrobidism, craniostenosis, other neoplasms, Hand-Schuller-Christian disease, and orbital hemorrhage due to trauma. Surgical resection is performed in cases with unilateral optic nerve involvement, the surgical approach being determined by tumor location. Bilateral or chiasmal cases are treated with radiotherapy when progression occurs. Malignant optic nerve gliomas and optic nerve hyperplasia are also discussed. Optic nerve meningiomas arise from the nerve sheath and are to be distinguished from orbital meningiomas arising from ectopic arachnoidal cells or those secondarily involving the orbit by extension from adjacent sites. Up to 80% of orbital meningiomas occur in females, in two age peaks, 25% in the first decade, and the rest in the 5th decade. Meningiomas present with visual loss and may produce proptosis, papilledema and/or optic atrophy, retinal striae, opticociliary shunts, limitation of extra-ocular movements, and lid edema, Signs of von Recklinghausen's disease should be sought. X-rays are the mainstay of diagnosis. Orbital meningiomas are composed of cells in sheets or in whorls with some spindle shaped cells. Calcifications are typical. Usually the dura is penetrated and the orbit invaded. Primary orbital meningiomas are locally infiltrating but do not metastasize. Complete local excision en bloc is recommended...

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Optic Nerve Glioma and Cerebellar Astrocytoma in a Patient With Von Recklinghausen'S Neurofibromatosis

Cited by 10 publications

References 8 publications

Cerebellar gliomas in children with NF1: pathology and surgery

Cerebellar gliomas in children with NF1: pathology and surgery

Plexiform neurofibroma affecting the upper parietal scalp, with cerebellar hamartoma: role of histopathology, colour Doppler imaging and magnetic resonance imaging

Tumors of the optic nerve

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