Chiasmatic
optic glioma is a rare
tumor with an erratic natural history, usually seen in young children. A prior study from this institution demonstrated that these lesions were frequently lethal, despite initial clinical stabilization following
radiation therapy, and that visual, intellectual, and late endocrinological disabilities were prevalent. A novel approach was developed in 1977, when an initial clinical response to
vincristine was recorded in a child with a recurrent
optic glioma. Since then, all children with recurrent
optic glioma and all children aged 6 years old and under with newly diagnosed
optic glioma have been offered a program of initial
therapy with
vincristine and
actinomycin D for six cycles over 18 months. The four children with recurrent
tumor who were treated with that regimen remain clinically stable 13
to 115 months after
chemotherapy. Twelve children (eight under 24 months old) with newly diagnosed
optic glioma have been treated with this program, and three are still on
therapy. Four developed progression while on
therapy, and five remain stable from 1 to 60 months posttherapy. The four children who developed progressive disease have been treated with
radiation therapy and remain stable. Six of the 12 children showed shrinkage of their
tumor on computerized tomography while receiving
chemotherapy. This program may serve as an alternative to initial
radiation therapy in young children.