LGG-20. CLINICAL FEATURES AND TREATMENT RESULTS FOR PEDIATRIC OPTICO-HYPOTHALAMIC ASTROCYTOMA

Current consensus for the treatment of optico-hypothalamic astrocytoma (OHA) is a chemotherapy-first policy, limiting the role of surgery for histopathological diagnosis and partial decompression. However, a subgroup of OHA patients show resistance to chemotherapy and have a worse prognosis. In this study, we retrospectively analyzed our clinical experiences of the treatment of patients with OHA in two university hospitals. We have extracted and analyzed the medical charts of 15 pediatric OHA patients treated in two university hospitals since 1990. NF-1-associated OHA patients were excluded. Patient ages ranged from 10 months to 21 years (median 7 years). Out of 15 cases, 12 patients had a tumor larger than 3 cm and classified as Dodge 3. The final histopathological diagnosis was pilocytic astrocytoma in 13 cases. Three patients with tumors classified as Dodge 1 or 2 show good prognosis only by biopsy or partial resection. However, regarding Dodge 3 tumor, patient prognosis is worse regardless of chemotherapy and radiotherapy. After the initial surgery, chemotherapy was administered in 11 cases and radiotherapy in 5 cases. Multiple surgeries are needed for tumor control in 7 patients. Four patients died of tumor progression or treatment-associated complications. When the initial tumor is large enough to cause neurological deterioration, a chemotherapeutic tumor suppressive effect might be limited in a subset of large OHA cases. Therefore, it is important to consider the proper timing of safe surgical decompression in the early phase when a large tumor does not respond to chemotherapy.