title: Long-term results of the AIEOP LNH-97 protocol for childhood lymphoblastic lymphoma.
abstract: BACKGROUND
  Treatment intensification was considered a suitable strategy to increase the cure rate of lymphoblastic lymphoma (LBL) in children.
  
  
  METHODS
  The AIEOP LNH-97 trial was run between 1997 and 2007 for newly diagnosed LBL in patients aged less than 18 years. Treatment schedule was based on the previous, LSA2-L2 derived, AIEOP LNH-92 protocol. Modifications included: increased dose of upfront cyclophosphamide and methotrexate, use of l-Asparaginase during induction therapy, intensive block therapy for slow responders, and late intensification ("Reinduction") for patients with advanced stage disease. Total therapy duration was 12 months for stage I and II, and 24 months for stage III and IV. Central nervous system prophylaxis did not include cranial irradiation.
  
  
  RESULTS
  114 eligible patients were enrolled, 84 males and 30 females; median age was 9 years. Complete remission was obtained in 98% of patients. After a median follow-up time of seven years, 29 patients failed due to progression of disease (n = 2), relapse (n = 25), or second malignancy (n = 2). The 7-year overall survival was 82% (standard error [SE] 4%) and the 7-year event-free survival was 74% (SE 4%). No subgroup showed significantly different event free survival. None of the patients died of front line chemotherapy-related toxicity.
  
  
  CONCLUSIONS
  Treatment intensification was associated with good outcome in children and adolescents with LBL, with limited toxicity. Prognosis after relapse was better for patients who underwent allogeneic hematopoietic stem cell transplantation. Measurements of biological markers and treatment response are necessary for achieving further improvement through more accurate identification and stratification of patients at risk of disease relapse.
authors.affiliation: Clinic of Pediatric Hemato-Oncology, Department of Women's and Children's Health, University of Padova, Padova, Italy.
authors.forename: Marta
authors.initials: M
authors.lastname: Pillon
authors.affiliation: Azienda Sanitaria Provinciale, Ragusa, Italy.
authors.forename: Maurizio
authors.initials: M
authors.lastname: Aricò
authors.affiliation: Istituto di Ricerca Pediatrica, Fondazione Citt, à, della Speranza, Padova, Italy.
authors.forename: Lara
authors.initials: L
authors.lastname: Mussolin
authors.affiliation: Clinic of Pediatric Hemato-Oncology, Department of Women's and Children's Health, University of Padova, Padova, Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Carraro
authors.affiliation: Clinica Pediatrica, Università degli Studi di Milano-Bicocca, Azienda Ospedaliera San Gerardo, Monza, Italy.
authors.forename: Valentino
authors.initials: V
authors.lastname: Conter
authors.affiliation: Clinica Pediatrica, Università degli Studi di Milano-Bicocca, Azienda Ospedaliera San Gerardo, Monza, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Sala
authors.affiliation: Pediatric Oncology Department, Santobono-Pausilipon Hospital of Napoli, Napoli, Italy.
authors.forename: Salvatore
authors.initials: S
authors.lastname: Buffardi
authors.affiliation: Department of Hematology-Oncology, Istituto Giannina Gaslini, Genova, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Garaventa
authors.affiliation: Department of Oncology, Pediatric Hematology and Oncology Unit, A.R.N.A.S. Ospedali Civico Di Cristina e Benfratelli, Palermo, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: D'Angelo
authors.affiliation: Pediatric Hematology-Oncology, Policlinico di Catania, Catania, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Lo Nigro
authors.affiliation: Division of Paediatric Haematology-Oncology, Department of Pediatrics, Bari, Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Santoro
authors.affiliation: Division of Pediatric Onco-Hematology, Regina Margherita Children's Hospital, Torino, Italy.
authors.forename: Matilde
authors.initials: M
authors.lastname: Piglione
authors.affiliation: Dipartimento di Onco-Ematologia Pediatrica, Ospedale Bambino Gesù, Roma, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Lombardi
authors.affiliation: Oncology-Haematology and BMT Unit, Ospedale dei Bambini, Spedali Civili, Brescia, Italy.
authors.forename: Fulvio
authors.initials: F
authors.lastname: Porta
authors.affiliation: Pediatric Hematology-Oncology, Azienda Ospedaliera Universitaria Integrata Verona, Verona, Italy.
authors.forename: Simone
authors.initials: S
authors.lastname: Cesaro
authors.affiliation: Hematology, Department of Cellular Biotechnologies and Hematology, Roma, Italy.
authors.forename: Maria L
authors.initials: ML
authors.lastname: Moleti
authors.affiliation: Pediatric Oncology Service,, Pediatric Department, Second University of Napoli, Napoli, Italy.
authors.forename: Fiorina
authors.initials: F
authors.lastname: Casale
authors.affiliation: Pediatric Hematology-Oncology, Ospedale Pediatrico Microcitemico, Cagliari, Italy.
authors.forename: Rossella
authors.initials: R
authors.lastname: Mura
authors.affiliation: Department of Pathology, San Bortolo Hospital, Vicenza, Italy.
authors.forename: Emanuele S G
authors.initials: ES
authors.lastname: d'Amore
authors.affiliation: Clinic of Pediatric Hemato-Oncology, Department of Women's and Children's Health, University of Padova, Padova, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Basso
authors.affiliation: Clinic of Pediatric Hemato-Oncology, Department of Women's and Children's Health, University of Padova, Padova, Italy.
authors.forename: Angelo
authors.initials: A
authors.lastname: Rosolen
mesh_terms: D000970:Antineoplastic Agents / Q000009:adverse effects / Q000627:therapeutic use*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use; D001215:Asparaginase / Q000009:adverse effects / Q000627:therapeutic use*; D014408:Biomarkers, Tumor / Q000097:blood; D002648:Child; D003520:Cyclophosphamide / Q000009:adverse effects / Q000627:therapeutic use*; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D060828:Induction Chemotherapy; D008297:Male; D008727:Methotrexate / Q000009:adverse effects / Q000627:therapeutic use*; D009364:Neoplasm Recurrence, Local; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy*; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D014408:Biomarkers, Tumor; D003520:Cyclophosphamide; D001215:Asparaginase; D008727:Methotrexate
keywords: childhood; long-term outcome; lymphoblastic lymphoma; non-Hodgkin lymphoma; treatment