2014+中枢神经系统原发性生殖细胞肿瘤的管理指南(第1版).pdfVIP

CLINICAL PRACTICE GUIDELINE CNS-010 version 1 MANAGEMENT OF PRIMARY GERM CELL TUMOURS OF THE CENTRAL NERVOUS SYSTEM Effective Date: July, 2014 The recommendations contained in this guideline are a consensus of the Alberta Provincial Central Nervous System Tumour Team synthesis of currently accepted approaches to management, derived from a review of relevant scientific literature. Clinicians applying these guidelines should, in consultation with the patient, use independent medical judgment in the context of individual clinical circumstances to direct care. guide.medlive.cn CLINICAL PRACTICE GUIDELINE CNS-010 version 1 BACKGROUND Germ cell tumours (GCTs) of the central nervous system (CNS) are rare; they account for 0.5 percent of all primary brain tumours.1 Most patients with CNS GCTs are children and young adults, with approximately 90 percent of cases occurring before the age of 20.1 GCTs most commonly present in the second decade of life. However, nongerminomatous GCTs (NGGCTs) tend to occur in younger children; incidence declines after the age of 30 years.2 GCTs of the CNS show a regional distribution, with higher frequency in Asian countries. Males have a significantly higher rate of CNS GCTs than females2 and a 15:1 incidence in the pineal region compared to females.12 Primary CNS GCTs share histological, genetic and therapeutic similarities to extracranial primary GCTs. CNS GCTs comprise two distinct histologic groupings: germinoma