title: AFP/beta-HCG secreting CNS germ cell tumors: long-term outcome with respect to initial symptoms and primary tumor resection. Results of the cooperative trial MAKEI 89.
abstract: OBJECTIVE
  The aim of the present study was to evaluate survival and factors influencing long-term outcome of patients with AFP/beta-HCG secreting (non-seminomatous) central nervous system germ cell tumors (secCNSGCT), who were prospectively collected in the cooperative MAKEI (German: maligne Keimzelltumoren) 89 protocol.
  
  
  METHODS
  Between January 1989 and January 1994, 28 patients with secCNS GCT were registered and treated according to the MAKEI 89 protocol. The protocol recommended, after a clinically or histologically proven diagnosis and cisplatin-based chemotherapy, a resection of residual tumor and craniospinal irradiation (30 Gy) with a tumor boost (20 Gy).
  
  
  RESULTS
  The estimated (Kaplan-Meier) event-free survival (EFS) of protocol patients is 0.57 +/- 0.09 (n = 28) and the relapse-free survival (RFS) is 0.67 +/- 0.10 (at five and ten years). With respect to long-term survival, the combination of marked neurological symptoms at diagnosis along with primary tumor resection seem to be the main negative prognostic risk factors (Fisher exact test p < 0.05). CNS dissemination at diagnosis can also be considered as a negative risk factor as 3 of 5 patients with primary dissemination died of the disease.
  
  
  CONCLUSIONS
  Cisplatin-based three agent chemotherapy followed by resection of the residual tumor and craniospinal irradiation (CSI) with tumor boost is a successful and well-tolerated treatment for secCNSGCTs. The possibility of a clinical diagnosis based on MRI and tumor markers together with the use of modern neurosurgical techniques gives us the chance to postpone or even avoid major surgery. This gives an additional chance to reduce acute morbidity and further decrease late effects.
authors.affiliation: Department of Pediatric Hematology and Oncology Children's Hospital, Heinrich Heine University Medical Center, University of Düsseldorf, Düsseldorf, Germany. calaminus@med.uni-duesseldorf.de
authors.forename: G
authors.initials: G
authors.lastname: Calaminus
authors.forename: M
authors.initials: M
authors.lastname: Bamberg
authors.forename: D
authors.initials: D
authors.lastname: Harms
authors.forename: H
authors.initials: H
authors.lastname: Jürgens
authors.forename: R D
authors.initials: RD
authors.lastname: Kortmann
authors.forename: N
authors.initials: N
authors.lastname: Sörensen
authors.forename: O D
authors.initials: OD
authors.lastname: Wiestler
authors.forename: U
authors.initials: U
authors.lastname: Göbel
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols; D014408:Biomarkers, Tumor; D016543:Central Nervous System Neoplasms / Q000188:drug therapy / Q000401:mortality* / Q000503:physiopathology / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D002945:Cisplatin / Q000627:therapeutic use; D003071:Cognition / Q000502:physiology; D003131:Combined Modality Therapy / Q000379:methods; D016371:Cranial Irradiation; D005069:Evaluation Studies as Topic; D005260:Female; D018237:Germinoma / Q000188:drug therapy / Q000401:mortality* / Q000503:physiopathology / Q000601:surgery*; D001556:Hexachlorocyclohexane / Q000378:metabolism; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D011379:Prognosis; D011446:Prospective Studies; D012189:Retrospective Studies; D015996:Survival Rate; D013995:Time; D013997:Time Factors; D016896:Treatment Outcome; D000509:alpha-Fetoproteins / Q000378:metabolism
chemical_list: D000970:Antineoplastic Agents; D014408:Biomarkers, Tumor; D000509:alpha-Fetoproteins; D001556:Hexachlorocyclohexane; D002945:Cisplatin; C023888:beta-hexachlorocyclohexane