Background: Data on management of gray zone lymphoma (GZL) in children and adolescents are scarce.
Procedure: This retrospective study assessed clinical characteristics and outcome in six Austrian patients with GZL less than 18 years of age (male-to-female ratio: 1:1; median age: 15.8 years).
Results: Two patients each had a classical Hodgkin lymphoma (cHL)-like and composite GZL subtype, and one patient each had a large B-cell non-Hodgkin lymphoma (LBCL)-like and sequential GZL subtype. All had advanced disease with mediastinal and extranodal involvement. Five patients received an LBCL- and one patient a cHL-directed polychemotherapy ± radiotherapy. Out of the former patients, three survived, including two who relapsed and underwent high-dose chemotherapy with autologous stem cell rescue. The latter patient survived.
Conclusions: GZL remains a diagnostic and therapeutic challenge, necessitating the development of novel treatment concepts performed in a prospective setting.
Keywords: checkpoint inhibitors; gray zone lymphoma; outcome; relapse; treatment.
© 2020 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals, Inc.