Long-term results of AIEOP LNH-92 protocol for the treatment of pediatric lymphoblastic lymphoma: a report of the Italian Association of Pediatric Hematology and Oncology.

BACKGROUND: Lymphoblastic lymphoma (LBL) is the second most frequent lymphoma subtype in childhood. It is commonly treated according to therapy strategies for lymphoblastic leukemia.
METHODS: The AIEOP LNH-92 protocol was a modified LSA2-L2 therapy used for both T- and B-cell precursor LBL and included Induction, Consolidation, and Maintenance treatment with a total duration of 11 and 24 months for stages I and II, stages III and IV disease, respectively.
RESULTS: Fifty-five eligible patients were enrolled, 40 males and 15 females, with a median age of 8 years. Complete remission was achieved in 93% of the cases. With a median follow-up of 9 years the event-free survival (EFS) was 69% and overall survival 72%. EFS of localized disease was 100%. The most frequent grades III and IV toxicity was hematologic and hepatic (elevated transaminases) toxicity. No toxic death nor second tumor were observed. Outcome was comparable to most concomitant international protocols for LBL, but inferior to recent trials that included reinduction treatment or a higher intensity therapy for high stage disease.
CONCLUSIONS: AIEOP LNH92 protocol demonstrated similar efficacy compared to contemporary regimens, with limited toxicity. Nevertheless, an intensified treatment is warranted for high stage disease.