Optic glioma warranting treatment in children

Abstract

Purpose To describe cases of optic pathway glioma (OPG) warranting treatment in children. Methods This is a retrospective review of pediatric patients treated for OPG. The clinical data and imaging studies were obtained from the medical records and radiology files of patients seen at the Pediatric NeuroOphthalmology Clinic at the University of Illinois, Chicago and the private office of the author (LMK). Results A total of seven cases with an age range of 3-48 months at presentation were reviewed. Three of the patients were also ultimately diagnosed with neurofibromatosis type 1. Presenting symptoms included proptosis, decreased vision, gaze deficit, and nystagmus. Four patients underwent biopsies that confirmed OPG. Six of the patients were treated with intravenous chemotherapy, with three patients requiring a second chemotherapy cycle. One patient was successfully treated with an en-bloc optic nerve excision. Two patients underwent unilateral enucleation owing to globe complications. Conclusion Although benign tumours, OPG can behave very aggressively in young children. Both chemotherapy and en-bloc excision can be employed for treatment.

Neoplasms Associated with Germline and Somatic NF1 Gene Mutations

“…However, the use of alkylating agents and multidrug regimens in children with NF1 runs the theoretical risk for the development of secondary malignancies [169,171,172]. Between 31% and 42.8% of tumors fail to respond to chemotherapy, with treatment failure more common among the posterior OPGs [173,174]. Radiation therapy is mainly indicated for progressive or recurrent OPGs in patients Ͼ10 years of age [169].…”

How to Handle Benign Optic Glioma of Childhood: A Short Overview

“…removal of the tumour, means sacrificing visual function. 12,13 This is therefore not viable if there is useful vision left. It may be adequate to remove an orbital glioma causing problematic proptosis if the eye is nearly blind.…”

Neurofibromatosis Type I: Genetics and Clinical Manifestations

“…Prompt neuro-imaging should be performed if concerning signs or symptoms develop (King et al, 2003;Sylvester et al, 2006). Treatment is generally reserved for patients with progressive visual loss and usually consists of some combination of radiation therapy, chemotherapy or surgical excision (Sylvester et al, 2006;Kaufman & Doroftei, 2006). …”