BackgroundThe management and prognostic factors of tectal glioma (TG) remain ambiguous, because it is an extremely rare neoplasm that occurs predominantly in the pediatric population. The objective of this study was to evaluate the risk factors for progression-free survival (PFS) in TG patients after ETV operation, elucidate the radiological features of TG, and propose a treatment protocol.MethodsFrom 2002 to 2018, 50 patients that preoperative imaging manifestations were low-grade TGs were treated at our institute. Clinical features, treatments, radiologic findings, biopsies, and pertinent risk factors were evaluated.ResultsA total of 50 patients with a diagnosis of TG were identified. Twenty-six (52%) patients were males. The median age at diagnosis was 11.5 years (range 0.5–19 years). All patients had symptoms related to obstructive hydrocephalus and were treated with endoscopic third ventriculostomy (ETV). After a median follow-up duration of 59 months (range 11.0–208.0 months), progression occurred in six patients (12%), with a median PFS time of 18.0 months (range 4.0–56.0 months). Twelve patients (24%) underwent a biopsy, one patient (8.3%) was diagnosed with anaplastic oligodendroglioma, one patient (8.3%) was diagnosed with astrocytoma (WHO grade II-III), five patients (41.7%) were diagnosed with pilocytic astrocytoma, and the type of tumor could not be confirmed in five patients (41.7%) due to the small amount of tumor sample, thus, these patients were diagnosed with gliosis. PFS rates at 1 and 5 years were 91.2% ± 4.2% and 84.9% ± 5.9%, respectively. A multivariate model demonstrated that a large tumor size and cystic changes are risk factors for progression.ConclusionETV has been uniformly successful in the management of hydrocephalus caused by TG. A large tumor size and cystic changes are risk factors for progression. Under the condition of safety, a biopsy should be performed. For patients with low-grade TG, ETV is often the only surgical procedure that most patients require.HighlightsTectal gliomas are generally low-grade gliomas with a favorable prognosis.The only surgical procedure that most patients with tectal glioma require is ETV.Under the condition of safety, neuroendoscopy for a pathological diagnosis should be performed.A large tumor size and cystic changes are risk factors for progression.