Department of Neurology, Memorial Sloan Kettering Cancer Center, New York, New York.
Department of Neurology, Weill Cornell Medicine, New York, New York.
JAMA. 2023 Feb 21;329(7):574-587. doi: 10.1001/jama.2023.0023.
IMPORTANCE: Malignant primary brain tumors cause more than 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 approximately 36%. OBSERVATIONS: Approximately 49% of malignant brain tumors are glioblastomas, and 30% are diffusely infiltrating lower-grade gliomas. Other malignant brain tumors include primary central nervous system (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 when compared with radiotherapy alone (2-year survival, 27.2% vs 10.9%; 5-year survival, 9.8% vs 1.9%; hazard ratio [HR], 0.6 [95% CI, 0.5-0.7]; P < .001). In patients with anaplastic oligodendroglial tumors with 1p/19q codeletion, probable 20-year overall survival following radiotherapy without vs with the combination of procarbazine, lomustine, and vincristine was 13.6% vs 37.1% (80 patients; HR, 0.60 [95% CI, 0.35-1.03]; P = .06) in the EORTC 26951 trial and 14.9% vs 37% in the RTOG 9402 trial (125 patients; HR, 0.61 [95% CI, 0.40-0.94]; P = .02). Treatment of primary CNS lymphoma includes high-dose methotrexate-containing regimens, followed by consolidation therapy with myeloablative chemotherapy and autologous stem cell rescue, nonmyeloablative chemotherapy regimens, or whole brain radiation. CONCLUSIONS AND RELEVANCE: The incidence of primary malignant brain tumors is approximately 7 per 100 000 individuals, and approximately 49% of primary malignant 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.
重要性：恶性原发性脑肿瘤每年导致美国超过 15000 人死亡。原发性恶性脑肿瘤的年发病率约为每 10 万人中有 7 例，并且随年龄增长而增加。五年生存率约为 36%。
观察结果：约 49%的恶性脑肿瘤为胶质母细胞瘤，30%为弥漫浸润性低级别胶质瘤。其他恶性脑肿瘤包括原发性中枢神经系统（CNS）淋巴瘤（7%）和恶性室管膜瘤（3%）和脑膜瘤（2%）。恶性脑肿瘤的症状包括头痛（50%）、癫痫发作（20%-50%）、认知障碍（30%-40%）和局灶性神经功能缺损（10%-40%）。钆基造影剂前后的磁共振成像（MRI）是评估脑肿瘤的首选影像学方法。诊断需要考虑组织病理学和分子特征的肿瘤活检。治疗因肿瘤类型而异，通常包括手术、化疗和放疗的组合。对于胶质母细胞瘤患者，与单独放疗相比，替莫唑胺联合放疗可提高生存率（2 年生存率，27.2%比 10.9%；5 年生存率，9.8%比 1.9%；风险比[HR]，0.6[95%CI，0.5-0.7]；P<.001）。在 EORTC 26951 试验中，对于携带 1p/19q 缺失的有丝分裂性少突胶质细胞瘤患者，在未接受与接受丙卡巴肼、洛莫司汀和长春新碱联合治疗的情况下，接受放疗后的 20 年总生存率分别为 13.6%和 37.1%（80 例患者；HR，0.60[95%CI，0.35-1.03]；P=.06），在 RTOG 9402 试验中，分别为 14.9%和 37%（125 例患者；HR，0.61[95%CI，0.40-0.94]；P=.02）。原发性中枢神经系统淋巴瘤的治疗包括大剂量甲氨蝶呤为基础的方案，随后进行骨髓清除性化疗和自体干细胞解救、非骨髓清除性化疗方案或全脑放疗的巩固治疗。
结论和相关性：原发性恶性脑肿瘤的发病率约为每 10 万人中有 7 例，其中约 49%的原发性恶性脑肿瘤为胶质母细胞瘤。大多数患者死于进行性疾病。胶质母细胞瘤的一线治疗是手术联合放疗和烷化剂替莫唑胺。
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