髓母细胞瘤是儿童时期最常见的恶性脑肿瘤,占该年龄群所有脑肿瘤的 15% 至 20%,,占所有颅后窝肿瘤的 30% 至 40%,并且在美国发病率为每年每百万人群约 6 例。 世界标准化发病率男童为每年每百万 5.5 例,女童为每年每百万 3.4 例。[4]Alston RD, Newton R, Kelsey A, et al. Childhood medulloblastoma in northwest England 1954 to 1997: incidence and survival. Dev Med Child Neurol. 2003 May;45(5):308-14.https://onlinelibrary.wiley.com/doi/epdf/10.1111/j.1469-8749.2003.tb00401.xhttp://www.ncbi.nlm.nih.gov/pubmed/12729144?tool=bestpractice.com 在丹麦和英格兰进行的研究提示发病率正在下降,[5]Agerlin N, Gjerris F, Brincker H, et al. Childhood medulloblastoma in Denmark 1960-1984: A population-based retrospective study. Childs Nerv Syst. 1999 Jan;15(1):29-36.http://www.ncbi.nlm.nih.gov/pubmed/10066017?tool=bestpractice.com[6]Thorne RN, Pearson AD, Nicoll JA, et al. Decline in incidence of medulloblastoma in children. Cancer. 1994 Dec 15;74(12):3240-4.http://www.ncbi.nlm.nih.gov/pubmed/7982188?tool=bestpractice.com[7]Chidambaram B, Santhosh V, Shankar SK. Identical twins with medulloblastoma occurring in infancy. Childs Nerv Syst. 1998 Sep;14(9):421-5.http://www.ncbi.nlm.nih.gov/pubmed/9808250?tool=bestpractice.com 虽然来自监测、流行病学和结果数据库 (SEER) 项目的数据提示发病率增加。[8]McNeil DE, Coté TR, Clegg L, et al. Incidence and trends in pediatric malignancies medulloblastoma/primitive neuroectodermal tumor: a SEER update. Surveillance Epidemiology and End Results. Med Pediatr Oncol. 2002 Sep;39(3):190-4.http://www.ncbi.nlm.nih.gov/pubmed/12210449?tool=bestpractice.com 症状表现的高峰年龄在 3 岁和 8 岁之间。[9]Pizzo P, Poplack D. Tumors of the central nervous system. In: Strother D, Pollack I, Fisher P, et al, eds. Principles and practice of pediatric oncology. 4th ed. Philadelphia, PA: Lippincott, Williams and Wilkins; 2002. 男性患者比例略微偏高 (1.5:1),并且有一些报告双胞胎出现家族性的髓母细胞瘤。[9]Pizzo P, Poplack D. Tumors of the central nervous system. In: Strother D, Pollack I, Fisher P, et al, eds. Principles and practice of pediatric oncology. 4th ed. Philadelphia, PA: Lippincott, Williams and Wilkins; 2002. 髓母细胞瘤有在脑脊液循环通路中转移的倾向,证据显示诊断时 11% 至 43% 的病例出现此类转移扩散。[10]Bailey CC, Gnekow A, Wellek S, et al. Prospective randomised trial of chemotherapy given before radiotherapy in childhood medulloblastoma. International Society of Paediatric Oncology (SIOP) and the (German) Society of Paediatric Oncology (GPO): SIOP II. Med Pediatr Oncol. 1995 Sep;25(3):166-78.http://www.ncbi.nlm.nih.gov/pubmed/7623725?tool=bestpractice.com[11]Allen JC, Epstein F. Medulloblastoma and other primary malignant neuroectodermal tumors of the CNS. The effect of patients' age and extent of disease on prognosis. J Neurosurg. 1982 Oct;57(4):446-51.http://www.ncbi.nlm.nih.gov/pubmed/7108593?tool=bestpractice.com Chang 系统用于转移性疾病的分类。[12]Chang CH, Housepian EM, Herbert C. An operative staging system and a megavoltage radiotherapeutic technic for cerebellar medulloblastomas. Radiology. 1969 Dec;93(6):1351-9.http://www.ncbi.nlm.nih.gov/pubmed/4983156?tool=bestpractice.com