title: Ewing-like sarcomas with BCOR-CCNB3 fusion transcript: a clinical, radiological and pathological retrospective study from the Société Française des Cancers de L'Enfant.
abstract: BACKGROUND
  This retrospective multicenter study assessed the clinical, radiological and pathological presentation, treatment and outcome of 26 patients with Ewing-like sarcoma harboring BCOR-CCNB3 gene fusion transcript. Tumor samples had been collected between 1994 and April 2012.
  
  
  METHODS
  Eligibility criteria included assessment of a BCOR-CCNB3 transcript-positive tumor after molecular analysis and availability of minimal clinical and pathological data. Radiological data were also retrieved when possible. Data were analyzed by descriptive statistics and methods for survival analysis.
  
  
  RESULTS
  Median age at diagnosis was 13.1 years (5.9 to 25.6 years). Most patients (24/26) had localized tumors. All tumors but five were localized to bone. CCNB3 immunochemistry showed strong nuclear staining on all samples. No specific radiological features were found. Most patients received chemotherapy (15 according to protocols designed for Ewing tumors), before and/or after local treatment (surgery and/or radiotherapy, with 46.2% receiving both). Local and metastatic relapses were of poor prognosis. Induction chemotherapy and treatment according to an Ewing protocol might influence survival for patients with localized tumors. Sixteen patients are alive in complete remission with a median follow-up of 86 months. Five year overall survival and disease-free survival were respectively 76.5% (95% CI, 58%-95%) and 67.9% (95% CI, 48%-88%).
  
  
  CONCLUSIONS
  BCOR-CCNB3 transcript-positive Ewing-like sarcoma diagnosis should be discussed for a transcript-negative small round cell sarcoma in a child, adolescent or young adult patient. Diagnosis needs to be stated through CCNB3 immunochemistry or transcript identification. The exquisite chemosensitivity of these tumors should encourage the use of polychemotherapy for appropriate care, associated with best local tumor control.
authors.affiliation: INSERM U830, Institut Curie - Research Center, 26 rue d'Ulm, Paris, Cedex 5, France.
authors.forename: Sarah
authors.initials: S
authors.lastname: Cohen-Gogo
authors.forename: Cécile
authors.initials: C
authors.lastname: Cellier
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Coindre
authors.forename: Véronique
authors.initials: V
authors.lastname: Mosseri
authors.forename: Gaëlle
authors.initials: G
authors.lastname: Pierron
authors.forename: Cécile
authors.initials: C
authors.lastname: Guillemet
authors.forename: Antoine
authors.initials: A
authors.lastname: Italiano
authors.forename: Laurence
authors.initials: L
authors.lastname: Brugières
authors.forename: Daniel
authors.initials: D
authors.lastname: Orbach
authors.forename: Valérie
authors.initials: V
authors.lastname: Laurence
authors.forename: Olivier
authors.initials: O
authors.lastname: Delattre
authors.forename: Jean
authors.initials: J
authors.lastname: Michon
mesh_terms: D000293:Adolescent; D000328:Adult; D001859:Bone Neoplasms / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology*; D002648:Child; D003131:Combined Modality Therapy; D019926:Cyclin B / Q000235:genetics*; D005260:Female; D005500:Follow-Up Studies; D005602:France; D050939:Gene Fusion; D006801:Humans; D008175:Lung Neoplasms / Q000000981:diagnostic imaging / Q000235:genetics / Q000556:secondary*; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D011518:Proto-Oncogene Proteins / Q000235:genetics*; D011859:Radiography; D012097:Repressor Proteins / Q000235:genetics*; D012189:Retrospective Studies; D012512:Sarcoma, Ewing / Q000000981:diagnostic imaging / Q000235:genetics / Q000556:secondary*; D015996:Survival Rate; D055815:Young Adult
chemical_list: C411984:BCOR protein, human; C503694:CCNB3 protein, human; D019926:Cyclin B; D011518:Proto-Oncogene Proteins; D012097:Repressor Proteins
keywords: BCOR protein; Ewing sarcoma; adolescent; cyclin B3 protein; fusion oncogene protein; small cell sarcoma