Medulloblastomas and central nervous system primitive neuroectodermal tumors.
Medulloblastomas and central nervous system primitive neuroectodermal tumors.
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DOI：
10.1007/s11864-003-0050-7
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发表时间：
2003-12-01
影响因子：
4.3
通讯作者：
McLean, Thomas W
中科院分区：
文献类型：
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作者：
McLean, Thomas W
髓母细胞瘤和原始神经外胚层肿瘤的治疗在过去三十年中取得了重大进展。最大手术切除是治疗的主要方法。然而，与许多其他中枢神经系统肿瘤不同，髓母细胞瘤和原始神经外胚层肿瘤对放疗和化疗有反应。尽管有这种反应，但这些肿瘤患者的预后仍然不稳定，在婴儿和转移性疾病患者中预后相对较差。这些肿瘤最常见于儿童，因此大多数临床试验强调减少长期后遗症，以及提高生存率。所有符合条件的新诊断患者都应参与临床试验。如果患者不符合条件或拒绝同意临床试验，目前最好的治疗方法是手术切除，然后每周一次放射治疗（3岁以下儿童除外）。对于高危患者，应给予36 Gy的颅脊髓照射，并向后窝/原发肿瘤床和大块转移性疾病部位增加19.8 Gy的照射。对于中等风险的患者，颅脊柱照射剂量可降低至23.4 Gy +后窝/肿瘤床32.4 Gy。放疗后，所有患者均应进行强化多模式化疗。
Significant advances in the treatment of medulloblastoma and primitive neuroectodermal tumors have been made in the past three decades. Maximal surgical resection is a mainstay of therapy. However, unlike many other central nervous system neoplasms, medulloblastoma and primitive neuroectodermal tumors are radiation and chemotherapy responsive. Despite this response, the prognosis for patients with these tumors remains variable and is relatively poor in infants and patients with metastatic disease. These tumors most commonly arise in children, thus most clinical trials emphasize the reduction of long-term sequelae, in addition to improving survival. All newly diagnosed patients who are eligible should be offered participation in a clinical trial. If a patient is ineligible or declines consent/assent for a clinical trial, the best current treatment approach is surgical resection, followed by radiation therapy (except for children younger than 3 years) with weekly vincristine. For high-risk patients, 36 Gy of craniospinal irradiation should be delivered plus a boost of 19.8 Gy to the posterior fossa/primary tumor bed and sites of bulk metastatic disease. For average-risk patients, the craniospinal irradiation dose may be lowered to 23.4 Gy plus 32.4 Gy to the posterior fossa/tumor bed. After radiation therapy, intensive multimodal chemotherapy should be used for all patients.