Prognostic significance of p80 and visceral involvement in childhood CD30 anaplastic large cell lymphoma (ALCL)

Background. Between 1976 and 1998, CD30+anaplastic large cell lymphoma (ALC L) was diagnosed in 44 children (28 males, 16 females, age range 2.7-16.1 y ears, median 10). Up to 1993, 32 such children were treated according to a common chemotherapeutic protocol that stratified patients according to stag e, without considering presenting features. Thereafter, patients presenting with visceral (lung, spleen, liver, gastro-intestinal tract) or mediastina l involvement were assigned to a high-risk treatment protocol with inductio n intensification. The generation of these two risk-groups was the result o f 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-2 2 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% (9 4% at 6 years) for group B, respectively. Procedure. We tested 15/21 cases of group A, arid 18/22 of group B for p80 immunoreactivity in order to inve stigate a possible correlation between ALCL locations and NPM-ALK expressio n. Results. Thirteen of 15 specimens in group A and 17/18 in group B were p ositive for p80. Conclusions. It is impossible to conclude anything about p 80 positivity based on a series of 33/44 patients with childhood ALCL, neit her 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. (C) 2001 Wiley-Liss, Inc.