Data on the spectrum of second malignant neoplasms (SMNs) after primary childhood non-Hodgkin's lymphoma (NHL) are scarce. One-hundred-and-eighty-nine NHL patients diagnosed in a 30 years period of 1980-2010 developing an SMN were retrieved from 19 members of the European Intergroup for Childhood NHL and/or the international Berlin-Frankfurt-Münster Study Group. Five subgroups of SMNs were identified: (1) myeloid neoplasms (n = 43; 23%), (2) lymphoid neoplasms (n = 51; 27%), (3) carcinomas (n = 48; 25%), (4) central nervous system (CNS) tumors (n = 19; 10%), and (5) "other" SMNs (n = 28; 15%). In 37 patients (20%) preexisting disorders were reported with 90% having any kind of cancer predisposition syndrome (CPS). For the 189 primary NHL patients, 5-year overall survival (OS) after diagnosis of an SMN was 56 ± 4%, being worst for patients with preexisting disorders at 28 ± 8%. Five-year OS rates were 38 ± 8%, 59 ± 7%, 79 ± 8%, 34 ± 12%, and 62 ± 11%, respectively, for patients with myeloid and lymphoid neoplasms, carcinomas, CNS tumors, and "other" SMNs (p < 0.0001). Patients with SMNs after childhood NHL having a reported CPS, mostly mismatch repair disorders, carried a very poor prognosis. Moreover, although outcome was favorable in some subtypes of SMNs after childhood NHL (carcinomas, lymphoid neoplasms), other SMNs such as myeloid neoplasms and CNS tumors had a dismal prognosis.

Belarusian Research Center for Pediatric Oncology Hematology and Immunology Minsk Belarus

Children's Cancer Center National Center for Child Health and Development Tokyo Japan

Department of Oncology P and A Kyriakou Children's Hospital Athens Greece

Department of Pediatric and Adolescent Oncology Gustave Roussy Cancer Center Paris Saclay University Villejuif France

Department of Pediatric Hematology and Oncology Charles University and University Hospital Motol Prague Czech Republic

Department of Pediatric Hematology and Oncology Federal Center for Pediatric Hematology Oncology and Immunology Moscow Russia

Department of Pediatric Hematology and Oncology Rheinisch Westfälische Technische Hochschule Aachen University Aachen Germany

Department of Pediatric Hematology and Oncology Schneider Children's Medical Center of Israel Petah Tikva Israel

Department of Pediatric Hematology and Oncology Semmelweis University Budapest Hungary

Department of Pediatric Hematology and Oncology St Anna Children's Hospital Medical University of Vienna Vienna Austria

Department of Pediatric Hematology and Oncology The Queen Silvia's Hospital for Children and Adolescents University of Gothenburg Gothenburg Sweden

Department of Pediatric Hematology and Oncology University Hospital Hamburg Eppendorf Hamburg Germany

Department of Pediatric Hematology and Oncology University Hospital Leuven Leuven Belgium

Department of Pediatric Hematology and Oncology University La Fe Hospital Valencia Spain

Department of Pediatric Hematology and Oncology University of Padova Padova Italy

Department of Pediatrics and Adolescent Medicine Li Ka Shing Faculty of Medicine The University of Hong Kong Queen Mary Hospital Pokfulam Hong Kong

Division of Pediatrics Hematology and Oncology University Medical Center Ljubljana Ljubljana Slovenia

Pediatric Hematology and Oncology University Hospital of Münster Münster Germany

Pediatric Hematology Oncology Research Laboratory and Pediatric Hematology Oncology Unit Division of Pediatrics Department Woman Mother Child Lausanne University Hospital Lausanne Switzerland

Princess Maxima Center for Pediatric Oncology Utrecht The Netherlands

Sackler Faculty of Medicine Tel Aviv University Tel Aviv Israel

Wolfson Childhood Cancer Center Newcastle University Newcastle UK

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Genomic abnormalities of TP53 define distinct risk groups of paediatric B-cell non-Hodgkin lymphoma