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 (ALCL) was diagnosed in 44 children (28 males, 16 females, age range 2.7-16.1 years, median 10). Up to 1993, 32 such children were treated according to a common chemotherapeutic protocol that stratified patients according to stage, without considering presenting features. Thereafter, patients presenting with visceral (lung, spleen, liver, gastro-intestinal tract) or mediastinal involvement were assigned to a high-risk treatment protocol with induction intensification. The generation of these two risk-groups was the result of 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-22 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% (94% at 6 years) for group B, respectively.

Procedure: We tested 15/21 cases of group A, and 18/22 of group B for p80 immunoreactivity in order to investigate a possible correlation between ALCL locations and NPM-ALK expression.

Results: Thirteen of 15 specimens in group A and 17/18 in group B were positive for p80.

Conclusions: It is impossible to conclude anything about p80 positivity based on a series of 33/44 patients with childhood ALCL, neither 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.