Surgical Management and Prognostic Factors Based on 50 Tectal Plate Gliomas

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.

Tectal gliomas: assessment of malignant progression, clinical management, and quality of life in a supposedly benign neoplasm

OBJECTIVETectal gliomas constitute a rare and inhomogeneous group of lesions with an uncertain clinical course. Because these supposedly benign tumors are frequently followed up by observation over many years, the authors undertook this analysis of their own case series in an effort to demonstrate that the clinical course is highly variable and that there is a potential for a progressive biology.METHODSClinical data analysis of 23 cases of tectal glioma (involving 9 children and 14 adults) was performed retrospectively. Radiographic data were analyzed longitudinally and MR images were evaluated for tumor volume, contrast enhancement, and growth progression. Quality of life was assessed using the EORTC BN20 and C30 questionnaires during follow-up in a subgroup of patients.RESULTSThe patients’ mean age at diagnosis was 29.2 years. The main presenting symptom at diagnosis was hydrocephalus (80%). Six patients were treated by primary tumor resection (26.1%), 3 patients underwent biopsy followed by resection (13.1%), and 3 patients underwent biopsy only (13.1%). For additional treatment of hydrocephalus, 14 patients (60.9%) received shunts and/or endoscopic third ventriculostomy. Radiographic tumor progression was observed in 47.9% of the 23 cases. The mean time between diagnosis and growth progression was 51.5 months, and the mean time to contrast enhancement was 69.7 months. Histopathological analysis was obtained in 12 cases (52.2%), resulting in 5 cases of high-grade glioma (3 cases of glioblastoma multiforme [GBM], grade IV, and 2 of anaplastic astrocytoma, grade III), 5 cases of pilocytic astrocytoma, 1 diffuse astrocytoma, and 1 ganglioglioma. Malignant progression was observed in 2 cases, with 1 case progressing from a diffuse astrocytoma (grade II) to a GBM (grade IV) within a period of 13 years. Quality-of-life measurements demonstrated distinct functional deficits compared to a healthy sample as well as glioma control cohorts.CONCLUSIONSAnalysis of this case series shows that a major subpopulation of tectal gliomas show progression and malignant transformation in children as well as in adolescents. These tumors therefore cannot be considered inert lesions and require histological confirmation and close follow-up. Quality-of-life questionnaires show that tectal glioma patients might benefit from special psychological support in emotional, social, and cognitive functionality.