Background: Optic pathway and hypothalamic gliomas (OPHGs) are challenging to surgically remove owing to their anatomical relationship. We previously reported on surgical treatment outcomes over a 10-year time frame. The purpose of this study was to update the OPHG clinical outcomes for cases in which chemotherapy has become the primary treatment option. The role of surgery was also revisited.
Methods: Patients with a diagnosis of OPHG who underwent treatment at Seoul National University Children's Hospital from February 1999 to July 2019 were included. A multidisciplinary approach was used to determine the patients' treatment plans. Chemotherapy was the first-line treatment for all patients. When symptoms of hydrocephalus existed, debulking surgery was performed to reopen the flow of cerebrospinal fluid.
Results: The study included 47 patients with OPHGs. The mean age was 6.9 years. Neurofibromatosis 1 was diagnosed in 3 patients. The extent of removal was none or biopsy in 13 (28%) cases, partial resection in 23 (49%) cases, and subtotal to gross total resection in 11 (23%) cases. In 32 (68%) patients, chemotherapy was first-line treatment. Ascites after ventriculoperitoneal shunt occurred in 3 cases, and 2 cases were successfully managed with debulking surgery. Treatment outcomes showed a 5-year overall survival rate of 97.7% and a 5-year progression-free survival rate of 47.7%.
Conclusions: OPHG management using less invasive operations and chemotherapy as first-line treatment is feasible. Debulking surgery in patients with OPHGs may be considered in cases with cerebrospinal fluid pathway obstruction, progression despite chemotherapy or radiation, and refractory shunt-related ascites.
Keywords: Ascites; Hypothalamic glioma; Optic pathway glioma; Pediatric; Pilocytic astrocytoma.
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