Prognostic factors in childhood anaplastic large cell lymphoma: Long term results of the international alcl99 trial

European Inter-Group for Childhood Non-Hodgkin lymphoma (EICNHL)

Research output: Contribution to journalArticlepeer-review

Abstract

With the aim of describing the long-term follow-up and to define the prognostic role of the clinical/pathological/molecular characteristics at diagnosis for childhood, adolescent and young adults affected by anaplastic large cell lymphoma (ALCL), we analyzed 420 patients aged up to 22 years homogeneously treated within the international ALCL99 trial. The 10-year progression free survival (PFS) was 70% and overall survival was 90%, rare late relapses occurred but no secondary malignancies were reported. Among clinical/pathological characteristics, only patients presenting a small cell/lymphohistiocytic (SC/LH) pattern were independently associated with risk of failure (hazard ratio = 2.49). Analysis of minimal disseminated disease (MDD), available for 162 patients, showed that both SC/LH pattern (hazard ratio = 2.4) and MDD positivity (hazard ratio = 2.15) were significantly associated with risk of failure in multivariate analysis. Considering MDD and SC/LH results, patients were separated into three biological/pathological (bp) risk groups: a high-risk group (bpHR) including MDD-positive patients with SC/LH pattern; a low-risk group (bpLR) including MDD-negative patients without SC/LH pattern; and an intermediate-risk group (bpIR) including remaining patients. The 10-year PFS was 40%, 75% and 86% for bpHR, bpIR and bpLR, respectively (p < 0.0001). These results should be considered in the design of future ALCL trials to tailor individual treatments.

Original languageEnglish
Article number2747
Pages (from-to)1-16
Number of pages16
JournalCancers
Volume12
Issue number10
DOIs
Publication statusPublished - Oct 2020

Bibliographical note

Funding Information:
Funding: This research was funded by Fondazione CA.RI.PA.RO, Padova, Italy (Grant 17/03) and AIRC, Milano, Italy (Investigator Grant—IG 2018 #21385) to LM. This work was also supported by Fondazione Giacomo Ascoli and Comitato Assistenza Socio-sanitaria in Oncoematologia Pediatrica (CASOP), by St. Anna Kinderkrebsforschung (St. Anna Children’s Cancer Research Institute), by the KindeKrebs Initiative Buchholz, Holm-Seppensen (KKI), and Cancer Research UK.

Publisher Copyright:
© 2020 by the authors. Licensee MDPI, Basel, Switzerland.

Keywords

  • ALCL
  • Childhood
  • Long-term follow-up
  • MDD

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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