Bevacizumab May Bring Back Vision in Optic Pathway Gliomas

November 21, 2013

By Will Boggs, MD

NEW YORK (Reuters Health) Nov 21 - Bevacizumab treatment of children with optic pathway gliomas (OPG) appears to produce marked recovery of vision, according to a new report of four cases.

"Bevacizumab provides a fast, robust response in these children," Dr. Robert A. Avery from Children's National Medical Center, Washington, DC told Reuters Health by email. "Although it's not first-line treatment, bevacizumab is a good alternative or adjunct treatment for children with OPG who are experiencing rapid visual loss or tumor progression."

OPGs, low-grade gliomas of the proximal afferent visual pathway, often cause vision loss that is largely unresponsive to standard treatment.

In a paper in JAMA Ophthalmology, online November 14, Dr. Avery and colleagues report marked recovery of visual acuity or visual field in four cases of pediatric OPGs treated with bevacizumab. The children included an 11-year-old girl with neurofibromatosis type 1 (NF1) and chiasmatic OPG; a 13-year-old girl with a sporadic OPG; a nine-year-old girl with an NF1-related chiasmatic OPG; and a six-year-old girl with a sporadic chiasm/hypothalamic OPG.

The 11-year-old girl presented with a dense temporal visual field loss that improved significantly within seven months of bevacizumab treatment and continued to improve and remained stable six months after treatment was discontinued.

The 13-year-old girl had reduced visual acuity (20/125) and a left hemianopsia. Her visual field improved after two months of bevacizumab monotherapy, and her visual acuity returned to normal after four months of therapy and remained stable at 12 months.

The nine-year-old girl's visual acuity improved from 20/400 to 20/100 within six weeks of bevacizumab treatment and remained stable for nine months after she stopped the treatment.

The six-year-old girl experienced improvements in visual acuity after starting bevacizumab. Nine months after discontinuation of the drug, her vision remained stable despite an increase in size and enhancement of her lesion.

Three children had previously been treated with bevacizumab-based treatment, but each experienced significant visual improvement with retreatment, the investigators say, "suggesting that response to bevacizumab may occur regardless of previous exposure."

"Given that most patients with OPG-related visual impairment will show modest or no visual improvement with standard chemotherapy, the incorporation of bevacizumab in these cases may greatly improve visual outcomes and should be considered in appropriate clinical situations," the researchers conclude. "Confirmation is warranted and will require prospective evaluation involving standardized and detailed ophthalmologic assessment."

"Treatment must be tailored to each child, but bevacizumab treatment will likely be continued for about 12 months unless there is no response or we encounter significant side effects," Dr. Avery said.

"It's not the be all and end all for these kids, but rather a temporizing measure," he concluded. "Most of these children will be treated in the context of a controlled trial."

Dr. Avery H. Weiss from Seattle Children's Hospital, who has studied OPG in children but was not part of the new research, said there is mounting evidence suggesting bevacizumab should be used to treat glioma.

"Increased understanding of glioma biology and objective measure of treatment effects provided by basic research scientists and clinical investigators have resulted in more effective, targeted therapies and improved outcomes," he told Reuters Health by email.

"On the basis of these data," Dr. Weiss concluded, "this reviewer contends that bevacizumab may show treatment efficacy in combination with chemotherapy or protein beam therapy but there is no evidence that it should replace conventional therapy."


JAMA Ophthalmol 2013.


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