Radiation-induced cerebral vasculopathy in children with neurofibromatosis and optic pathway glioma

Grill, Jacques; Couanet, D.; Cappelli, Carlo; Habrand, J.L.; Rodriguez, Diana; Sainte‐Rose, Christian; Kalifa, Chantal

doi:10.1002/1531-8249(199903)45:3<393::aid-ana17>3.0.co;2-b

Cited by 221 publications

(99 citation statements)

References 19 publications

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“…17 Rapamycin has been shown to cause regression of astrocytomas in tuberous sclerosis. 18 Although traditional radiotherapy is contraindicated as it leads to a poorer neuropsychological outcome, 19 second tumours, and vascular complications, 20 the Mayo Clinic is currently running a phase II study of the use of conformal radiotherapy. 21 Only by understanding the natural history of the disease in more depth will we know how best to use these new emerging treatments.…”

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confidence: 99%

Screening children with NF1 for optic pathway glioma – Yes

Simmons

Gogi

2010

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confidence: 99%

Screening children with NF1 for optic pathway glioma – Yes

Simmons

Gogi

2010

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“…Of the tumors involving the chiasm, 33-60% are considered posterior lesions that also involve the hypothalamus or third ventricle. 15,22,30,33 Approximately 75% of these tumors are diagnosed during the first decade of life and 60% are diagnosed before the age of 5 years, which also portends a less favorable prognosis. 2,29,33,74,83 One of the most complete reviews of all cases of OPG was published in 1994 by Dutton,15 who retrospectively reviewed all cases of OPG reported in the literature up to 1992, which included 2297 patients.…”

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confidence: 99%

Optic pathway gliomas: a review

Binning

Liu

Kestle

et al. 2007

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169

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✓Optic pathway gliomas represent approximately 3–5% of childhood intracranial tumors. They usually occur in children during the first decade of life and are seen in 11–30% of patients with neurofibromatosis Type 1 (NF1). Although these tumors are typically low-grade gliomas, the clinical course and natural history are highly variable, making treatment paradigms difficult. Overall, however, they are often indolent tumors that can be observed over time for progression without initial treatment, especially in patients with NF1. Chemotherapy is the first-line treatment for progressive tumors, and radiation therapy is reserved for patients with progressive disease who are older than 5–7 years. Surgery is reserved for large tumors causing mass effect or hydrocephalus and tumors confined to the orbit or unilateral optic nerve.

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“…Disease progression can be controlled by irradiation (Kovalic et al, 1990), but endocrinological and neurological late effects are severe (Cappelli et al, 1998). The principal sequelae of this tumour and its treatment consist of growth hormone deficiency and other endocrinological deficits (Brauner et al, 1990), cognitive deficits (Packer et al, 1983) and cerebrovascular complications (Grill et al, 1999). Moreover, when neurofibromatosis type 1 (NF1) is also present, one may encounter additional learning difficulties (Moore et al, 1994).…”

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confidence: 99%

Neuropsychological outcome in children with optic pathway tumours when first-line treatment is chemotherapy

et al. 2003

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Standard treatment of optic pathways gliomas consists of radiotherapy and surgery when feasible. Owing to the toxicity of irradiation, chemotherapy has emerged as an interesting therapeutic option, especially in young children. This study describes the neuropsychological profile of 27 children (aged between 1.5 and 15.7 years) with optic pathways gliomas treated with chemotherapy as first-line treatment. Eight of them also received radiotherapy as salvage treatment. Eight had neurofibromatosis type 1 (NF1). Intellectual outcome was preserved in children treated with chemotherapy only (mean ¼ 107717) compared to children also receiving radiotherapy (mean IQ ¼ 88724) or children having NF1 and treated with chemotherapy (mean IQ ¼ 80713). Scores for abstract reasoning, mental arithmetic, chessboard/coding, perception, judgement of line orientation were lower in children irradiated than in those treated only by chemotherapy. Children with Nf1 showed subnormal IQ scores with marked impairment of shortand long-term memory. With respect to long-term neuropsychological outcome, our study shows that a chemotherapy-first strategy can preserve the intellectual outcome of these patients either by avoiding the need of radiotherapy or by delaying its use as much as possible.

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Radiation-induced cerebral vasculopathy in children with neurofibromatosis and optic pathway glioma

Cited by 221 publications

References 19 publications

Screening children with NF1 for optic pathway glioma – Yes

Screening children with NF1 for optic pathway glioma – Yes

Optic pathway gliomas: a review

Neuropsychological outcome in children with optic pathway tumours when first-line treatment is chemotherapy

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