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.
