Optic pathway tumor in children: Toward a new classification for neurosurgical use

Neurosurgery for Optic Pathway Glioma: Optimizing Multidisciplinary Management

“…In addition to the side effects that are often associated with cytotoxic chemotherapy (e.g., myelosuppression, neurotoxicity, hypersensitivity reactions), progression rate on chemotherapy can be as high as 50% ( 37 ). Although gross total resection of OPG may not often be possible, significant operative debulking has demonstrated satisfactory outcomes in selected cases, and there exist clear indications for other surgical interventions (See section on Surgical Nuances) ( 4 – 6 , 8 , 12 , 13 , 18 , 19 , 38 ). Illustrating the variable opinions regarding the role of surgery for OPG, a consensus statement from 2011 suggested limiting the role of tissue biopsy to sporadic OPGs in relevant clinical trials or those with atypical radiographic features, all in the setting of multidisciplinary discussion ( 3 ).…”

“…Optic pathway glioma (OPG) can arise in the optics nerve(s), optic chiasm, optic tract, lateral geniculate body, and hypothalamus and is most typical in the chiasmatic/hypothalamic region ( 4 , 5 ). OPGs are diagnosed by the first decade of life in 75% and the second decade of life in 90% of patients ( 6 ). They can be sporadic or in association with Neurofibromatosis Type 1 (NF1).…”

“…Presentation results from involvement in or compression of the optic pathway and diencephalon, CSF obstruction from hydrocephalus, or mass effect from large tumors ( 8 ). Children may exhibit clinical signs of visual impairment, endocrine dysfunction, diencephalic syndrome (Russell syndrome), and life-threatening intracranial hypertension ( 2 , 5 , 6 , 8 ).…”

“…Complete surgical resection of OPG is generally not possible due to their location and complex anatomical relationship to sensitive structures. It is important to note that various opinions exist regarding surgical strategies for OPG ( 3 – 6 , 8 , 12 , 13 , 17 ). Broadly accepted surgical indications include management of hydrocephalus with CSF diversion (most commonly VP shunt), debulking for symptomatic/life-threatening mass effect, and drainage of symptomatic cystic components ( 5 , 6 , 8 , 11 , 12 , 18 , 19 ).…”

“…It is important to note that various opinions exist regarding surgical strategies for OPG ( 3 – 6 , 8 , 12 , 13 , 17 ). Broadly accepted surgical indications include management of hydrocephalus with CSF diversion (most commonly VP shunt), debulking for symptomatic/life-threatening mass effect, and drainage of symptomatic cystic components ( 5 , 6 , 8 , 11 , 12 , 18 , 19 ). Regardless, no treatment modality has surpassed conventional chemotherapy as the gold standard for tumor control in pediatric OPG.…”