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

Abstract

<jats:sec><jats:title>OBJECTIVE</jats:title><jats:p>Tectal 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.</jats:p></jats:sec><jats:sec><jats:title>METHODS</jats:title><jats:p>Clinical 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.</jats:p></jats:sec><jats:sec><jats:title>RESULTS</jats:title><jats:p>The 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.</jats:p></jats:sec><jats:sec><jats:title>CONCLUSIONS</jats:title><jats:p>Analysis 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.</jats:p></jats:sec>

Tectal glioma as a distinct diagnostic entity: a comprehensive clinical, imaging, histologic and molecular analysis

“…Pediatric TG should be considered a chronic disease, in which care for long-term morbidities is of paramount importance. Caregivers should be informed of the common long term morbidities in patients with TG including chronic headache, persistent visual symptoms and neurocognitive impairments [ 1 , 4 , 14 , 16 , 20 , 22 , 25 , 28 , 29 , 39 , 43 , 48 ]. Neuropsychologic assessments in our cohort suggested areas of deficit in working memory, processing speed and academics, specifically math, thus adding to prior reports of problems in visual attention deficits, behavior problems, and academic achievement, calling for neuropsychologic evaluation as standard of care in patients with TG [ 1 , 14 ].…”

Long-Term Outcomes of Patients with Hydrocephalus Secondary to Tectal Plate Glioma versus Idiopathic Aqueductal Stenosis: Results from a Single Center

“…Finally, the psychosocial impact of this disease should not be underestimated. Mohme et al [11] assessed the quality of life in 12 TPG patients using the EORTC-QLQ-C30 and EORTC-QLQ-BN20 questionnaires and found that patients reported significant impairments in social, cognitive, and emotional functioning compared to healthy controls. They argue that these differences were probably mediated by the overall psychological sequels of the diagnosis rather than functional impairment caused directly by the tumor, supported by the fact that "future uncertainty" was documented in 18% of the interviewed patients.…”

“…In contrast to most of the other brain stem tumors, they show a benign and indolent clinical course. Therefore, they have been considered a distinct entity among glial brain stem tumors [5][6][7][8][9][10][11][12][13]. However, their location may lead to compression of the cerebral aqueduct and often results in secondary hydrocephalus.…”

Evaluation of Radiosurgery Target Volume Definition for Tectal Gliomas with Incorporation of Magnetic Resonance Imaging (MRI): An Original Article

“…Given the indolent nature of these tumours, observation may be an option in selected patients. However, therapeutic options including surgical interventions, systemic agents, and radiation therapy (RT) have been utilized for management of tectal gliomas as upfront or salvage therapies [2,3,[9][10][11][12][13][14][15][16][17]. Contemporary RT techniques and radiosurgery in the forms of Stereotactic Radiosurgery (SRS), Stereotactic Body Radiotherapy (SBRT) and Hypo fractionated Stereotactic Radiation Therapy (HFSRT) may be utilized for focused irradiation of several central nervous system disorders as well as tumours located throughout the human body .…”