Article
Tectal gliomas: assessment of malignant progression, clinical management and quality of life in a supposedly benign neoplasm
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Published: | June 18, 2018 |
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Objective: Tectal gliomas are a rare and inhomogeneous group of lesions with an uncertain clinical course. Since these supposedly benign tumors, which are frequently followed up by observation over many years, we aim to show from our series, that the clinical course is highly variable and that there is a potential for a progressive biology.
Methods: Clinical data analysis of 23 patients with tectal gliomas (9 children, 14 adults) were performed retrospectively. Radiographic data were analyzed as a time-course and magnetic resonance imaging was evaluated for tumor volume, contrast enhancement and growth progression. Quality of life was assessed using the EORTC BN20 and C30 questionnaires during follow-up on a subgroup of patients.
Results: Eighteen patients underwent cranial surgery and 35 surgical procedures were performed, including nine (25.7%) resection and eight (22.9%) stereotactic or endoscopic biopsies, six shunts (17.1%) and twelve endoscopic ventriculostomies (34.3%). In 47.9% of all patients radiographic tumor progression was observed, with a mean time between diagnosis to growth progression of 51.5 months and a mean time to contrast enhancement of 69.7 months. Histopathological analysis was obtained only in 11 cases showing five cases of high grade gliomas (3 GBM °IV, 2 AA °III), five cases of pilocytic and one ganglioglioma. Malignant progression was observed in 2 cases, with one case progressing from a diffuse astrocytoma °II to a GBM °IV within a time-period of 13 years. Mean age at diagnosis was 29.2 years. Main presenting symptom at diagnosis was hydrocephalus (80%). Quality of life measurements demonstrated significant and distinct deficits in, e.g. emotional and social function compared to healthy as well as glioma control cohorts (p<0.05).
Conclusion: We demonstrate that tectal gliomas can show a fast progression and malignant transformation in children as well as in adolescents. The presence of several malignant cases is an argument to perform close follow-up in patients with histologically proven tectal glioma as they cannot be considered as a priori inert lesions, and carry the risk of rapid transformation. Quality of life questionnaires revealed that tectal glioma patients might benefit from special psychological support in emotional-, social- and cognitive functionality.