Surgical Options of Chiasmatic Hypothalamic Glioma - a Relevant Part of Therapy in an Interdisciplinary Approach for Tumor Control

Abstract

Objective Extent of resection of pediatric low-grade glioma mostly improves progression free survival. In chiasmatic hypothalamic glioma (CHG) complete resections are limited due to high risk of associated neurological and endocrinological deficits. Still, surgery has its role in the framework of a multidisciplinary team (MDT) approach. We report our retrospective experience from two centers on surgical options and their impact on long-term outcome. Methods Medical records of surgically treated pediatric CHG patients between 2004 to 2022 were analyzed. Patient characteristics, surgical interventions, histology, and non-surgical therapy were retrieved together with outcome measures such as visual acuity, endocrine function, and survival. Results A total of 63 patients (33 female, NF-1: n = 8) were included. Age at first diagnosis was 4.6 years (range: 0.2–16.9) and cohort follow-up was 108 ± 71 months. 20 patients were surgically treated with a biopsy and 43 patients with debulking at a median age of 6.5 years (range: 0.16–16.9). Patients received a median of 2 tumor surgeries (range: 1–5). Cyst drainage was accomplished in 15 patients and 27 patients had ventriculoperitoneal shunt implantation. Non-surgical therapy was given in 69.8%. At the end of follow up 74.6% of patients had stable disease. The cohort had a median Karnofsky score of 90 (range: 0-100). Four patients died. Hormone substitution was necessary in 30.2% and visual acuity was impaired in 66% of patients. Conclusion Pediatric CHG is a chronic disease due to overall high survival with multiple progressions. Surgical therapy remains a key treatment option offering biopsy, tumor-debulking, cyst fenestration and hydrocephalus management in the framework of MDT decision making. Team experience contributes to reduce possible deficits in this challenging cohort.