Epidemiology, diagnosis, and optimal management of glioma in adolescents and young adults

Abstract

Neoplasms of the central nervous system (CNS) are the most frequently encountered solid tumors of childhood, but are less common in adolescents and young adults (AYA), aged 15–39 years. Gliomas account for 29%–35% of the CNS tumors in AYA, with approximately two-thirds being low-grade glioma (LGG) and the remaining being high-grade glioma (HGG). We review the epidemiology, work-up, and management of LGG and HGG, focusing on the particular issues faced by the AYA population relative to pediatric and adult populations. Visual pathway glioma and brainstem glioma, which represent unique clinical entities, are only briefly discussed. As a general management approach for both LGG and HGG, maximal safe resection should be attempted. AYA with LGG who undergo gross total resection (GTR) may be safely observed. As age increases and the risk factors for recurrence accumulate, adjuvant therapy should be more strongly considered with a strong consideration of advanced radiation techniques such as proton beam therapy to reduce long-term radiation-related toxicity. Recent results also suggest survival advantage for adult patients with the use of adjuvant chemotherapy when radiation is indicated. Whenever possible, AYA patients with HGG should be enrolled in a clinical trial for the benefit of centralized genetic and molecular prognostic review and best clinical care. Chemoradiation should be offered to all World Health Organization grade IV patients with concurrent and adjuvant chemotherapy after maximal safe resection. Younger adolescents with GTR of grade III lesions may consider radiotherapy alone or sequential radiotherapy and chemotherapy if unable to tolerate concurrent treatment. A more comprehensive classification of gliomas integrating pathology and molecular data is emerging, and this integrative strategy offers the potential to be more accurate and reproducible in guiding diagnostic, prognostic, and management decisions.

Pediatric low-grade glioma in the era of molecular diagnostics

“…Tumors of the central nervous system (CNS) are the most frequent solid tumors in children, with approximately 5.4-5.6 diagnoses per 100,000 [48,154,155]. Of those diagnosed, 0.7 per 100,000 will succumb to their disease, making CNS tumors the leading cause of cancer related death in children [154,155,168].…”

Coronarin D Induces Apoptotic Cell Death and Cell Cycle Arrest in Human Glioblastoma Cell Line

“…However, stem-like cells within the CNS are now thought to be the cells of origin of several primary brain tumor types, including glioblastomas [5,6]. Gliomas are one of the most fatal tumors, presenting a high mortality rate, 29-35%, of the CNS tumors in adolescents and young adults [7,8]. Among gliomas, glioblastomas (GBM) are the most aggressive and frequent subtype [9,10].…”

Epidemiología y caracterización general de los tumores cerebrales primarios en el adulto

“…Entre las décadas tercera y cuarta son más frecuentes los oligodendrogliales y en las décadas sexta y séptima aumenta la incidencia de gliomas malignos. La ubicación anatómica más frecuente de las lesiones gliales es el lóbulo frontal (2,31).…”

“…El tratamiento estándar de los pacientes con glioblastoma es la realización de cirugía citorreductora máxima (si es posible), seguida de radiación (dosis aproximada 60 Gy dividida en 30 fracciones), en combinación con temozolomida; un agente de metilación oral. Con 6-12 meses adicionales de temozolomida posradiación, se evidenció una mejor respuesta a la quimiorradiación en pacientes que tienen la metilación del promotor de la enzima O6metilguanina-ADN metiltransferasa (MGMT) (2,6,30,31,32,33).…”