Non-anaplastic peripheral T cell lymphoma in children and adolescents—an international review of 143 cases

Abstract

Peripheral T cell lymphomas (PTCL) are rare in children and adolescents, and data about outcome and treatment results are scarce. The present study is a joint, international, retrospective analysis of 143 reported cases of non-anaplastic PTCL in patients <19 years of age, with a focus on treatment and outcome features. One hundred forty-three patients, between 0.3 and 18.7 years old, diagnosed between 2000 and 2015 were included in the study. PTCL not otherwise specified was the largest subgroup, followed by extranodal NK/T cell lymphoma, hepatosplenic T cell lymphoma (HS TCL), and subcutaneous panniculitis-like T cell lymphoma (SP TCL). Probability of overall survival (pOS) at 5 years for the whole group was 0.56 ± 0.05, and probability of event-free survival was (pEFS) 0.45 ± 0.05. Patients with SP TCL had a good outcome with 5-year pOS of 0.78 ± 0.1 while patients with HS TCL were reported with 5-year pOS of only 0.13 ± 0.12. Twenty-five percent of the patients were reported to have a pre-existing condition, and this group had a dismal outcome with 5-year pOS of 0.29 ± 0.09. The distribution of non-anaplastic PTCL subtypes in pediatric and adolescent patients differs from what is reported in adult patients. Overall outcome depends on the subtype with some doing better than others. Pre-existing conditions are frequent and associated with poor outcomes. There is a clear need for subtype-based treatment recommendations for children and adolescents with PTCL.

Incidence and outcomes of rare paediatric non‐hodgkin lymphomas

“…Patients with HSTCL had dismal outcomes. In this series, the 5‐year OS was only 9·6 ± 8·8%, confirming previous reports (Mellgren et al , ).…”

“…Paediatric patients with rare systemic forms of T cell NHL (PTCL, ENKL and HSTCL) had more guarded prognoses (Fig ). In this category, PTCL patients had the best outcome (5‐year OS 59·0 ± 5·1% and 10‐year OS 56·3 ± 5·5%), which was similar to a European cohort of paediatric PTCL patients (Mellgren et al , ). In children, the outcome is suggested to be dependent on disease staging (Hutchison et al , ; Windsor et al , ).…”

Non-Hodgkin lymphoma and pre-existing conditions: spectrum, clinical characteristics and outcome in 213 children and adolescents

“…43,45 Notably, 36 of 143 (25%) had a pre-existing condition and fared very poorly, with 5-year EFS rates of 11%±7%. 45 In summary, half of our patients suffered from an event, with half of these patients experiencing a relapse/progression; the other half died from therapy-related toxicity. Moreover, our study also discovered a male predominance in children and adolescents with pre-existing conditions and NHL (approx.…”

Treatment and prognosis of mature (non‐anaplastic) T‐ and NK‐cell lymphomas in childhood, adolescents, and young adults

“…Treatment outcome of patients with HSTL is very guarded (Mellgren et al , ; Sorge et al , ). HSTL is not responsive to anthracycline‐ or platinum‐based chemotherapy (Belhadj et al , ; McThenia et al , ).…”

“…In fact, haemophagocytic syndrome seems to be less common among paediatric cases. Among 20 cases of paediatric SPTCL reported by the European Intergroup for Childhood Non‐Hodgkin Lymphoma (EICNHL) and International Berlin‐Frankfurt‐Munster (i‐BFM) group, none presented or developed that complication (Mellgren et al , ).…”