title: Treatment Outcome and Prognostic Molecular Markers of Supratentorial Primitive Neuroectodermal Tumors.
abstract: BACKGROUND
  To identify prognostic factors and define the optimal management of patients with supratentorial primitive neuroectodermal tumors (sPNETs), we investigated treatment outcomes and explored the prognostic value of specific molecular markers.
  
  
  METHODS
  A total of 47 consecutive patients with pathologically confirmed sPNETs between May 1985 and June 2012 were included. Immunohistochemical analysis of LIN28, OLIG2, and Rad51 expression was performed and correlated with clinical outcome.
  
  
  RESULTS
  With a median follow-up of 70 months, 5-year overall survival (OS) and progression-free survival (PFS) was 55.5% and 40%, respectively, for all patients. Age, surgical extent, and radiotherapy were significant prognostic factors for OS and PFS. Patients who received initially planned multimodal treatment without interruption (i.e., radiotherapy and surgery (≥subtotal resection), with or without chemotherapy) showed significantly higher 5-year OS (71.2%) and PFS (63.1%). In 29 patients with available tumor specimens, tumors with high expression of either LIN28 or OLIG2 or elevated level of Rad51 were significantly associated with poorer prognosis.
  
  
  CONCLUSIONS
  We found that multimodal treatment improved outcomes for sPNET patients, especially when radiotherapy and ≥subtotal resection were part of the treatment regimen. Furthermore, we confirmed the prognostic significance of LIN28 and OLIG2 and revealed the potential role of Rad51 in sPNETs.
authors.affiliation: Departments of Radiation Oncology, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Seo Hee
authors.initials: SH
authors.lastname: Choi
authors.affiliation: Departments of Pathology, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Se Hoon
authors.initials: SH
authors.lastname: Kim
authors.affiliation: Departments of Neurosurgery, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Kyu-Won
authors.initials: KW
authors.lastname: Shim
authors.affiliation: Departments of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Jung Woo
authors.initials: JW
authors.lastname: Han
authors.affiliation: Department of Pharmacy, College of Pharmacy, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Junjeong
authors.initials: J
authors.lastname: Choi
authors.affiliation: Departments of Neurosurgery, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Dong-Seok
authors.initials: DS
authors.lastname: Kim
authors.affiliation: Departments of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Chuhl Joo
authors.initials: CJ
authors.lastname: Lyu
authors.affiliation: Department of Radiation Oncology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Jun Won
authors.initials: JW
authors.lastname: Kim
authors.affiliation: Departments of Radiation Oncology, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Chang-Ok
authors.initials: CO
authors.lastname: Suh
authors.affiliation: Departments of Radiation Oncology, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Jaeho
authors.identifier: http://orcid.org/0000-0001-9966-5157
authors.initials: J
authors.lastname: Cho
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D051792:Basic Helix-Loop-Helix Proteins / Q000378:metabolism; D014408:Biomarkers, Tumor / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000378:metabolism; D018242:Neuroectodermal Tumors, Primitive / Q000378:metabolism / Q000473:pathology / Q000628:therapy*; D000076104:Oligodendrocyte Transcription Factor 2; D011379:Prognosis; D016601:RNA-Binding Proteins / Q000378:metabolism; D051135:Rad51 Recombinase / Q000378:metabolism; D015173:Supratentorial Neoplasms / Q000378:metabolism / Q000473:pathology / Q000628:therapy*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D051792:Basic Helix-Loop-Helix Proteins; D014408:Biomarkers, Tumor; D009419:Nerve Tissue Proteins; D000076104:Oligodendrocyte Transcription Factor 2; D016601:RNA-Binding Proteins; D051135:Rad51 Recombinase; C488352:Lin-28 protein, mouse; C405708:OLIG2 protein, human; C495153:RAD51 protein, human