Follow-Up of Optic Pathway Gliomas in Children with Neurofibromatosis Type 1

Kuenzle, C.-H.; Weissert, M; Roulet, E.; Bode, Hans H.; Schefer, S.; Huisman, T.-H.; Landau, Klara; Boltshauser, Eugen

doi:10.1055/s-2008-1073043

Cited by 55 publications

(43 citation statements)

References 21 publications

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“…Four of the 43 patients with an intracranial tumour developed a second tumour during the follow-up period. More than half of the cohort described by Kuenzle et al [19]developed a second tumour during 9 years of follow-up, and Hochstrasser et al [11]and Sørensen et al [20]found second tumours in 21 and 47%, respectively, of children with NF. Direct comparison of these studies with our cohort is difficult because of the differences in follow-up periods.…”

Section: Discussionmentioning

confidence: 99%

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Growth Hormone and Neurofibromatosis

Howell

Wilton²,

Lindberg³

et al. 2000

Horm Res Paediatr

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Data collected from 102 neurofibromatosis (NF) children with growth hormone (GH) deficiency (GHD) who were receiving GH replacement therapy were reviewed to assess the efficacy and safety of GH therapy in this condition. GH was administered at a mean dose of 0.18 mg/kg/week. During the 1st year the median height velocity increased significantly from 4.2 cm/year before treatment to 7.1 cm/year, and the median height standard deviation score increased from –2.4 to –1.9. The response to therapy, however, was not as good as that observed in patients with idiopathic GHD. GH therapy did not influence the progression of any of the features of NF, including intracranial tumours, and was not associated with an excess of other adverse events. We conclude that GH treatment of NF patients with GHD is beneficial in terms of improved growth rate and is well tolerated.

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Section: Discussionmentioning

confidence: 99%

“…Kuenzle et al [19]described 21 such children, of whom 11 were found to have a cerebral tumour during a follow-up period of 9 years. Hochstrasser et al [11]studied a group of 133 NF children, of whom 24 had intracranial tumours, of which 20 were optic gliomas.…”

Section: Discussionmentioning

confidence: 99%

Growth Hormone and Neurofibromatosis

Howell

Wilton²,

Lindberg³

et al. 2000

Horm Res Paediatr

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“…But more than 50% of the patients have very indolent or no progression of the disease, thus they do not need treatment. A nonprogressive evolution is more likely to occur in children with NF1 [9]. This phakomatosis is present in up to 50% of children with OPHG.…”

Section: Discussionmentioning

confidence: 99%

Optic Pathway Hypothalamic Gliomas in Children under Three Years of Age: The Role of Chemotherapy

Silva¹,

Goldman²,

Keating³

et al. 2000

Pediatr Neurosurg

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Objectives: Optic pathway/hypothalamic gliomas (OPHGs) tend to occur in young children. Treatment options consist of surgical resection, radiation therapy (RT) and chemotherapy. Due to complications induced by surgery and RT, chemotherapy has gained significant recognition for the treatment of OPHG in young children. Chemosensitivity of OPHG in very young children under 3 years of age has not been well documented. We analyzed 14 patients who were treated with chemotherapy with or without surgery. Materials and Methods: Fourteen children younger than 3 years (median age of 10 months) with OPHG were treated between 1988 and 1998. Magnetic resonance imaging was obtained in all cases. Hydrocephalus was present in 8 patients and diencephalic syndrome was noted in 6. Only 3 of these had evidence of neurofibromatosis-1. Five patients had partial tumor resection and 4 had endoscopic biopsy at the time of ventriculoperitoneal shunt placement. Pathological examination revealed low-grade astrocytoma in 5 and juvenile pilocytic astrocytoma in 4. All patients received chemotherapy: carboplatin in 8, a combination of carboplatin and vincristine in 4 and a combination of other agents in 2. Results: Eight (57%) of 14 patients had a sustained reduction of tumor during the follow-up time between 15 months and 8 years. The 5-year progression-free survival was 63%. These tumor reductions were often accompanied by clinical improvements. Diencephalic syndrome responded to chemotherapy alone in 4 of 6 patients. However, 5 others had progressive disease; 3 during the treatment and 2 following the treatment (9 months and 2 years, respectively). All these 5 patients had a partial tumor resection prior to chemotherapy. Conclusion: A majority of OPHGs responds to chemotherapy. Due to slow progression of these tumors and adverse effects of other therapeutic modalities, we recommend chemotherapy as a primary treatment for OPHGs. Our present data indicates that partial surgical resection does not enhance chemotherapy effectiveness for OPHGs in infants or children younger than 3 years.

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“…35) Even partial resection of the tumor with palliative decompression of the eloquent structures can lead to regress of symptoms. 3,10) However, in cases of intrinsic chiasmatic gliomas the role of surgery is usually limited to verification of histologi cal diagnosis, 1,4,6,9,15) whereas chemotherapy and/or irradiation are considered to be the main treatment options. Such approaches can provide stabilization or improvement of vision in 88.993% of pa tients, 2,13) but complete restoration of visual func tions is very unusual.…”

Section: Discussionmentioning

confidence: 99%

Complete Recovery of Visual Functions After Multimodality Treatment for Intrinsic Chiasmatic-Hypothalamic Astrocytoma-Case Report-

Chernov

Ivanov

Zhinzhina

et al. 2004

Neurol. Med. Chir.(Tokyo)

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A 21yearold female was admitted with complaints of severe impairment of vision. The visual acuity was 0.02 in both eyes along with residual visual fields and central scotomas. Neuroimaging disclosed a chiasmatichypothalamic glioma. Surgery was performed with partial removal of the intrachiasmatic part of the intrinsic tumor. The histological diagnosis was fibrillary astrocytoma. Progressive recovery of vision began in the first week after surgery. Adjuvant treatment included one course of fractionated radiation therapy and six courses of chemotherapy. Complete recovery of visual acuity occurred after 10 months, and the visual fields were restored after an additional 6 months. Her vision has been stable during 2 years of follow up. The prognosis for recovery of vision after treatment of optic pathway gliomas mainly depends on the severity of visual loss at admission and is negatively influenced by intrinsic tumor growth, symmetrical extension, and involvement of the chiasm. Despite the presence of all these factors in the present case, multimodality management resulted in the complete recovery of visual functions. Surgery may be indicated in cases of intrinsic chiasmatic gliomas complicated by severe visual loss.

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Follow-Up of Optic Pathway Gliomas in Children with Neurofibromatosis Type 1

Cited by 55 publications

References 21 publications

Growth Hormone and Neurofibromatosis

Growth Hormone and Neurofibromatosis

Optic Pathway Hypothalamic Gliomas in Children under Three Years of Age: The Role of Chemotherapy

Complete Recovery of Visual Functions After Multimodality Treatment for Intrinsic Chiasmatic-Hypothalamic Astrocytoma-Case Report-

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