title: Prognostic factors in childhood anaplastic large cell lymphoma: results of a large European intergroup study.
abstract: To study prognostic factors of progression/relapse, data concerning 225 children enrolled between 1987 and 1997 in Berlin-Frankfurt-Münster, Société Française d'Oncologie Pédiatrique and United Kingdom Children's Cancer Study Group prospective studies for the treatment of anaplastic large cell lymphoma (ALCL) were merged. Median follow-up was 9.3 years. Five-year overall survival and event-free survival of the whole population was 81% (95% confidence interval, 76%-86%) and 69% (63%-74%), respectively. B symptoms, mediastinal involvement, skin lesions, visceral involvement, St Jude stage 3-4, Ann Arbor stage 3-4, and elevated lactate dehydrogenase increased the risk of progression/relapse in the univariate analysis. In the multivariate analysis, 3 factors remained significant: mediastinal involvement (relative risk [RR] = 2.1 [1.2-3.5]), visceral involvement defined as lung, liver, or spleen involvement (RR = 2.1 [1.3-3.6]), and skin lesions (RR = 1.9 [1.1-3.2]). Five-year progression-free survival (PFS) of the 81 patients with none of these risk factors was 89% [82%-96%], contrasting with a 5-year PFS of 61% [53%-69%] in the 144 patients with at least 1 risk factor (RR = 4.4 [2.2-8.9; P < .001). In conclusion, 3 factors associated with an increased risk of failure in childhood ALCL have been defined: mediastinal involvement, visceral involvement, and skin lesions.
authors.affiliation: Biostatistics and Epidemiology Unit, Institut Gustave-Roussy, Villejuif, France. le_deley@igr.fr
authors.forename: Marie-Cécile
authors.initials: MC
authors.lastname: Le Deley
authors.forename: Alfred
authors.initials: A
authors.lastname: Reiter
authors.forename: Denise
authors.initials: D
authors.lastname: Williams
authors.forename: Georges
authors.initials: G
authors.lastname: Delsol
authors.forename: Ilske
authors.initials: I
authors.lastname: Oschlies
authors.forename: Keith
authors.initials: K
authors.lastname: McCarthy
authors.forename: Martin
authors.initials: M
authors.lastname: Zimmermann
authors.forename: Laurence
authors.initials: L
authors.lastname: Brugières
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005060:Europe; D005260:Female; D006801:Humans; D007223:Infant; D017728:Lymphoma, Large-Cell, Anaplastic / Q000453:epidemiology* / Q000473:pathology*; D008297:Male; D011379:Prognosis; D012307:Risk Factors