TY - JOUR
T1 - Chemotherapy for optic nerve glioma in a child with neurofibromatosis type-1
AU - Parentin, Fulvio
AU - Rabusin, Marco
AU - Zennaro, Floriana
AU - Orth, Dario Catalano
AU - Pensiero, Stefano
PY - 2008/6
Y1 - 2008/6
N2 - The majority of optic pathway tumors associated with Neurofibromatosis type-1 (NF-1) are benign, slow-growing lesions: however, rapidly growing tumors, which cause proptosis and visual loss, can sometimes occur. Optimal management of these tumors is still unclear. We report the case of a 12-year-old male, affected by NF-1 who was found to have a rapidly growing right optic nerve glioma, treated with carboplatin and vincristine. During chemotherapy, optic disc swelling rapidly disappeared; the orbital tumor decreased in size, with magnetic resonance imaging (MRI) evidence of tumor shrinkage. There was a progressive improvement in visual acuity and Visually Evoked Potential (VEP) amplitudes. Children with NF-1 associated optic pathway tumors should not receive chemotherapy unless there is documented progressive disease. Nevertheless, in contrast with previous studies, this case demonstrates that chemotherapy can be a safe and effective treatment for NF-1-associated optic nerve glioma, and should not be reserved only for patients with optic chiasm or tract involvement.
AB - The majority of optic pathway tumors associated with Neurofibromatosis type-1 (NF-1) are benign, slow-growing lesions: however, rapidly growing tumors, which cause proptosis and visual loss, can sometimes occur. Optimal management of these tumors is still unclear. We report the case of a 12-year-old male, affected by NF-1 who was found to have a rapidly growing right optic nerve glioma, treated with carboplatin and vincristine. During chemotherapy, optic disc swelling rapidly disappeared; the orbital tumor decreased in size, with magnetic resonance imaging (MRI) evidence of tumor shrinkage. There was a progressive improvement in visual acuity and Visually Evoked Potential (VEP) amplitudes. Children with NF-1 associated optic pathway tumors should not receive chemotherapy unless there is documented progressive disease. Nevertheless, in contrast with previous studies, this case demonstrates that chemotherapy can be a safe and effective treatment for NF-1-associated optic nerve glioma, and should not be reserved only for patients with optic chiasm or tract involvement.
KW - Chemotherapy
KW - Glioma
KW - Neurofibromatosis
KW - Optic nerve
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U2 - 10.1080/01658100802115163
DO - 10.1080/01658100802115163
M3 - Article
AN - SCOPUS:45749153005
VL - 32
SP - 159
EP - 162
JO - Neuro-Ophthalmology
JF - Neuro-Ophthalmology
SN - 0165-8107
IS - 3
ER -