title: Long-term results of the first Italian Association of Pediatric Hematology and Oncology protocol for the treatment of pediatric B-cell non-Hodgkin lymphoma (AIEOP LNH92).
abstract: BACKGROUND
  Childhood B-cell lymphomas (B-NHLs) represent a group of aggressive malignancies that are amenable to high-intensity chemotherapy regimens. In 1992, the Italian Association of Pediatric Hematology and Oncology (AIEOP) initiated a prospective clinical trial involving the diagnosis and treatment of childhood B-NHL based on a well established strategy developed by the Berlin-Frankfurt-Munster Group.
  
  
  METHODS
  Between November 1992 and October 1997, 163 children who had B-NHL were treated prospectively in the first national AIEOP trial. Disease staging was performed according to the St. Jude staging system, and treatment was assigned on the basis of risk group (R1, R2, or R3), which took into account disease stage and resectability and serum lactate dehydrogenase (LDH) levels.
  
  
  RESULTS
  Of the 144 evaluable patients, 11 had Stage I disease, 35 had Stage II disease, 76 had Stage III disease, and 22 had Stage IV disease. Thirteen, 54, and 77 patients were included in risk groups R1, R2, and R3, respectively. The 10-year overall survival (OS) and event-free survival (EFS) rates for the overall population were 89.4% and 81.8%, respectively; the EFS rates for patients in risk groups R1, R2, and R3 were 100%, 86.9%, and 75.1%, respectively. Multivariate analysis indicated that age > or = 10 years, disease histology other than Burkitt or Burkitt-like lymphoma, and LDH levels > or = 1000 international units per liter had negative prognostic value. Analysis of the toxicity (according to the World Health Organization grading system) associated with 710 of the 748 chemotherapy cycles administered revealed 855 cases of Grade 3 or 4 toxicity, with 73% being cases of hematologic toxicity. Toxic episodes were most common after the first chemotherapy cycle and were equally common in the R2 and R3 risk groups. To date, the development of a second malignancy has not been observed in any patient in the study cohort.
  
  
  CONCLUSIONS
  Long-term follow-up of the current study (AIEOP LNH92) confirms the observation of a favorable outcome for patients with B-NHL treated with short, intensive chemotherapy regimens and raises the possibility that non-Burkitt or non-Burkitt-like histology and age > or = 10 years may have negative prognostic value for patients with childhood B-NHL.
authors.affiliation: Clinica di Oncoematologia Pediatrica, Azienda Ospedaliera-Università di Padova, Padua, Italy.
authors.forename: Marta
authors.initials: M
authors.lastname: Pillon
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Di Tullio
authors.forename: Alberto
authors.initials: A
authors.lastname: Garaventa
authors.forename: Simone
authors.initials: S
authors.lastname: Cesaro
authors.forename: Maria Caterina
authors.initials: MC
authors.lastname: Putti
authors.forename: Claudio
authors.initials: C
authors.lastname: Favre
authors.forename: Alma
authors.initials: A
authors.lastname: Lippi
authors.forename: Gianmarco
authors.initials: G
authors.lastname: Surico
authors.forename: Andrea
authors.initials: A
authors.lastname: Di Cataldo
authors.forename: Emanuele
authors.initials: E
authors.lastname: D'Amore
authors.forename: Luigi
authors.initials: L
authors.lastname: Zanesco
authors.forename: Angelo
authors.initials: A
authors.lastname: Rosolen
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007558:Italy; D016393:Lymphoma, B-Cell / Q000188:drug therapy*; D008297:Male; D009364:Neoplasm Recurrence, Local; D011379:Prognosis; D015996:Survival Rate; D016896:Treatment Outcome