Long-term clinical and visual outcomes after surgical resection of pediatric pilocytic/pilomyxoid optic pathway gliomas

Abstract

<jats:sec><jats:title>OBJECTIVE</jats:title><jats:p>The choice of treatment modality for optic pathway gliomas (OPGs) is controversial. Chemotherapy is widely regarded as first-line therapy; however, subtotal resections have been reported for decompression or salvage therapy as first- and second-line treatment. The goal of this study was to further investigate the role and efficacy of resection for OPGs.</jats:p></jats:sec><jats:sec><jats:title>METHODS</jats:title><jats:p>A retrospective chart review was performed on 83 children who underwent surgical treatment for OPGs between 1986 and 2014. Pathology was reviewed by a neuropathologist. Clinical outcomes, including progression-free survival (PFS), overall survival (OS), and complications, were analyzed.</jats:p></jats:sec><jats:sec><jats:title>RESULTS</jats:title><jats:p>The 5- and 10-year PFS rates were 55% and 46%, respectively. The 5- and 10-year OS rates were 87% and 78%, respectively. The median extent of resection was 80% (range 30%–98%). Age less than 2 years at surgery and pilomyxoid features of the tumor were found to be associated with significantly lower 5-year OS. No difference was seen in PFS or OS of children treated with surgery as a first-line treatment compared with children with surgery as a second- or third-line treatment. Severe complications included new disabling visual deficit in 5%, focal neurological deficit in 8%, and infection in 2%. New hormone deficiency occurred in 22% of the children.</jats:p></jats:sec><jats:sec><jats:title>CONCLUSIONS</jats:title><jats:p>Approximately half of all children experience a long-term benefit from resection both as primary treatment and as a second-line therapy after failure of primary treatment. Primary surgery does not appear to have a significant benefit for children younger than 2 years or tumors with pilomyxoid features. Given the risks associated with surgery, an interdisciplinary approach is needed to tailor the treatment plan to the individual characteristics of each child.</jats:p></jats:sec>

The Visual Acuity Outcome and Relevant Factors Affecting Visual Improvement in Pediatric Sporadic Chiasmatic–Hypothalamic Glioma Patients Who Received Surgery

“…Konovalov et al (30) studied 45 patients with giant OPG, all of whom received surgical treatment, and found that 54% of these patients had VA improvement at the last followup. Hidalgo et al (10) also reported OPG VA outcomes after first-or second-line surgical treatment and found that only 14% of the children had better vision. Our study found that 29.87% of vision-impaired eyes had VA improvement after primary intratumor partial resection.…”

“…The role of surgical treatment of the disease is to preserve VA, relieve intracranial pressure, reduce tumor burden, and control intractable progression (8,9). Although primary surgery showed good efficacy and safety for CHG patients in some studies (10,11), information on the effectiveness of surgery to improve visual acuity (VA) is limited. The identity of patients who are more likely to benefit from surgery, and the extent that is low risk and effective for decompression are not known.…”

Comparison of two surgical methods for the treatment of optic pathway gliomas in the intraorbital segment: an analysis of long-term clinical follow-up, which evaluates the surgical outcomes

“…Goodden et al (13) believe that the indications for surgery should include: (I) progressive deterioration of vision and eye exophthalmos, (II) huge, cystic tumors that make it difficult to reduce the space-occupying effect through chemotherapy, (III) serious tumor blockage caused by ventricular system hydrocephalus, and (IV) damage to the hypothalamic-pituitary endocrine axis, especially in infants. However, in recent years, many centers have explored surgical treatment of OPGs, and have achieved significant surgical results for intractable OPGs (14,15). Therefore, it is necessary to summarize the clinical characteristics, treatment principles, and prognosis of OPGs in order to better understand them and the effects of treatment.…”

A systematic review of ongoing clinical trials in optic pathway gliomas

“…The timing and nature of the surgical approaches to OPGs have not been subjected to rigorous trial data. Surgical options include biopsy, partial/subtotal resection and radical resection [13]. Furthermore, the optimal timing of chemotherapy/radiotherapy initiation and the influence this has on overall outcomes are not fully known.…”

“…This multifaceted care underlies the complexity of the disease. Advances in the understanding and treatment of OPG could come from a variety of sources, including novel or repurposed pharmacological agents, emerging biological agents and tumour vaccines, refinement of surgical approaches, diagnostic and therapeutic radiological developments or quality of lifefocussed research [10][11][12][13][14][15][16]. The aim of this systematic review was to formulate a comprehensive list of all current ongoing clinical trials that were specifically looking at a clinical care of OPGs in order to identify trends in current research and provide an overview of the field to guide future research efforts.…”