title: Recent advances in molecular biology and treatment strategies for intracranial germ cell tumors.
abstract: BACKGROUND
  Intracranial germ cell tumors (IGCTs) are a group of rare pediatric brain tumors which include various subtypes. The current understanding of the etiology of the tumors and their optimal management strategies remain controversial.
  
  
  METHODS
  The data on IGCTs were collected from articles published in the past 20 years, and the origin and etiology of IGCTs at molecular level as well as the relative roles of varied treatment strategies in different prognosis groups according to Matsutani's classification were reviewed.
  
  
  RESULTS
  Recent cellular and molecular evidence suggests that IGCTs may arise from the transformation of endogenous brain cells; and findings in the molecular characterization of IGCTs suggest roles of CCND2, RB1, and PRDM14 in the pathogenesis of IGCTs and identify the KIT/RAS and AKT1/mTOR pathways as potential therapeutic targets in future. According to Matsutani's classification of IGCTs, the good prognosis group includes both germinomas and mature teratomas. For germinomas, both radiation alone and reduced-dose radiotherapy in combination with adjuvant chemotherapy are effective, while complete surgical excision is recommended for mature teratomas. In the intermediate prognosis group, immature teratoma has been successfully treated with gamma knife surgery. However, for intermediate prognosis IGCTs other than immature teratomas, gross total resection with adjuvant chemotherapy and radiotherapy or gamma knife surgery may be necessary to achieve cure. In the poor prognosis group, survival outcomes are unsatisfactory, and complete surgical resection combined with more intensive chemotherapy and radiotherapy remains the best available treatment option at this time.
  
  
  CONCLUSIONS
  IGCTs should be strictly classified according to their pathological categories before administering pathology-specific treatments. Although open microsurgical excision is the traditional surgical strategy for IGCTs, recent publications also support the role of endoscopic surgical options for pineal region IGCTs.
authors.affiliation: Department of Neurosurgery, Huashan Hospital, Fudan University, 12 Wulumuqi Zhong Road, Shanghai, China.
authors.forename: Xiang
authors.initials: X
authors.lastname: Huang
authors.affiliation: Department of Neurosurgery, Huashan Hospital, Fudan University, 12 Wulumuqi Zhong Road, Shanghai, China. rong.z.zhang@gmail.com.
authors.forename: Rong
authors.initials: R
authors.lastname: Zhang
authors.affiliation: Department of Neurosurgery, Huashan Hospital, Fudan University, 12 Wulumuqi Zhong Road, Shanghai, China.
authors.forename: Ying
authors.initials: Y
authors.lastname: Mao
authors.affiliation: Department of Neurosurgery, Huashan Hospital, Fudan University, 12 Wulumuqi Zhong Road, Shanghai, China.
authors.forename: Liang-Fu
authors.initials: LF
authors.lastname: Zhou
authors.affiliation: Department of Neurosurgery, Huashan Hospital, Fudan University, 12 Wulumuqi Zhong Road, Shanghai, China.
authors.forename: Chao
authors.initials: C
authors.lastname: Zhang
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D059248:Chemoradiotherapy / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D004724:Endoscopy / Q000379:methods*; D005260:Female; D018237:Germinoma / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D008866:Microsurgery / Q000379:methods; D009361:Neoplasm Invasiveness / Q000473:pathology; D009367:Neoplasm Staging; D009373:Neoplasms, Germ Cell and Embryonal / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D011379:Prognosis; D016634:Radiosurgery / Q000379:methods; D035583:Rare Diseases; D018570:Risk Assessment; D016019:Survival Analysis; D013724:Teratoma / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D016896:Treatment Outcome
keywords: etiology; germ cell tumor; prognosis; treatment