Glioblastoma and other Primary Brain Malignancies in Adults. A Review

By L. Schaff, I. Mellinghoff. Journal of the American Medical Association (JAMA), 2023

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Importance: Malignant primary brain tumors cause over 15,000 deaths per year in the United States. The annual incidence of primary malignant brain tumors is approximately 7 per 100,000 individuals and increases with age. Five-year survival is about 67%. Observations: Malignant brain tumors include glioblastoma (49%), other gliomas with infiltration in the central nervous system (CNS) parenchyma (about 30%), primary CNS lymphoma (7%), and malignant forms of ependymomas (3%) and meningiomas (2%). Symptoms of malignant brain tumors include headache (50%), seizures (20–50%), neurocognitive impairment (30–40%), and focal neurologic deficits (10–40%). Magnetic resonance imaging before and after a gadolinium-based contrast agent is the preferred imaging modality for evaluating brain tumors. Diagnosis requires tumor biopsy with consideration of histopathological and molecular characteristics. Treatment varies by tumor type and often includes a combination of surgery, chemotherapy, and radiation. For patients with glioblastoma, the combination of temozolomide with radiotherapy improved survival compared to radiotherapy alone (2-year survival: 27.2% versus 10.9%; 5-year survival: 9.8% versus 1.9%)(HR 0.6, 95% CI 0.5–0.7; p<0.001). In patients with anaplastic oligodendroglial tumors with 1p/19q codeletion, probable 20-year overall survival following radiotherapy without versus with the combination of procarbazine, lomustine, and vincristine was 13.6% versus 37.1% (n=80; HR 0.60; 95% CI 0.35–1.03; p=0.06) in the EORTC 26951 trial and 14.9% versus 37% in the RTOG 9402 trial (n=125; HR 0.61; 95% CI 0.40–0.94; p=0.02). Treatment of primary CNS lymphoma includes high-dose methotrexate containing regimens, followed by consolidation therapy with myeloablative chemotherapy and autologous stem cell rescue, non-myeloablative chemotherapy regimens, or whole brain radiation. Conclusions and Relevance: The incidence of primary malignant brain tumors is about 7 per 100,000 individuals and approximately 49% of brain tumors are glioblastomas. Most patients die from progressive disease. First line therapy for glioblastoma is surgery followed by radiation and the alkylating chemotherapeutic agent temozolomide.

Subfields: Genetics, Neurology, Pulmonary and Respiratory Medicine

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