title: Prognostic significance of p80 and visceral involvement in childhood CD30 anaplastic large cell lymphoma (ALCL).
abstract: BACKGROUND
  Between 1976 and 1998, CD30+anaplastic large cell lymphoma (ALCL) was diagnosed in 44 children (28 males, 16 females, age range 2.7-16.1 years, median 10). Up to 1993, 32 such children were treated according to a common chemotherapeutic protocol that stratified patients according to stage, without considering presenting features. Thereafter, patients presenting with visceral (lung, spleen, liver, gastro-intestinal tract) or mediastinal involvement were assigned to a high-risk treatment protocol with induction intensification. The generation of these two risk-groups was the result of a retrospective analysis of clinical risk factors for therapy failure as previously reported [Massimino M, Gasparini M, Giardini R, Ann Oncol 1995;6:915-920]. Considering the whole cohort of patients divided into group A-21/22 evaluable patients with visceral/mediastinal involvement, and group B-22 evaluable patients, with other ALCL location-disease-free survival (DFS) and survival (S) at 5 years were 57 and 58% for group A, and 83 and 100% (94% at 6 years) for group B, respectively.
  
  
  METHODS
  We tested 15/21 cases of group A, and 18/22 of group B for p80 immunoreactivity in order to investigate a possible correlation between ALCL locations and NPM-ALK expression.
  
  
  RESULTS
  Thirteen of 15 specimens in group A and 17/18 in group B were positive for p80.
  
  
  CONCLUSIONS
  It is impossible to conclude anything about p80 positivity based on a series of 33/44 patients with childhood ALCL, neither about over-all prognosis nor about the role of visceral involvement. In adults, NPM-ALK protein expression is a favourable prognostic factor. Med Pediatr Oncol 2001;37:97-102.
authors.affiliation: Pediatric Oncology Unit, Istituto Nazionale Tumori, Via G. Venezian, I-20133 Milan, Italy. Massimino@istitutotumori.mi.it
authors.forename: M
authors.initials: M
authors.lastname: Massimino
authors.forename: F
authors.initials: F
authors.lastname: Spreafico
authors.forename: R
authors.initials: R
authors.lastname: Luksch
authors.forename: R
authors.initials: R
authors.lastname: Giardini
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D007118:Immunoassay; D007223:Infant; D017730:Ki-1 Antigen / Q000276:immunology*; D016403:Lymphoma, Large B-Cell, Diffuse / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D008297:Male; D009362:Neoplasm Metastasis; D011379:Prognosis; D011505:Protein-Tyrosine Kinases / Q000032:analysis / Q000096:biosynthesis*; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome; D014781:Viscera / Q000473:pathology
chemical_list: D017730:Ki-1 Antigen; C107023:p80(NPM-ALK) protein; D011505:Protein-Tyrosine Kinases