Non‐anaplastic peripheral t‐cell lymphoma in childhood and adolescence: A Children's Oncology Group study

Abstract

Background Peripheral T-cell lymphomas (PTCL) other than anaplastic large cell lymphoma are rare in young patients. While a high proportion of adults with PTCL have poor risk disease, pediatric PTCL is not well characterized. This study examines the outcome of localized and advanced PTCL in pediatric patients treated in standardized fashion. Procedure We identified 20 pediatric patients diagnosed with PTCL whose tumor cells did not express CD30 and/or ALK, as determined by immunohistochemistry, between 1992 and 2000 on one of two treatment protocols for localized NHL (POG 9219) or advanced stage large cell lymphoma (POG 9315). All cases were centrally reviewed. Results and Conclusions The median age was 12.6 (range 0.7 to 16.9) – 9 male and 11 female. Histological subtypes in the WHO Classification included PTCL, unspecified (12), extra-nodal NK/T-cell lymphoma of nasal type (4), sub-cutaneous panniculitis-like T cell lymphoma (1) and enteropathy-type T-cell lymphoma (1). 2 cases exhibited both T-cell and histiocyte markers and were reclassified as histiocytic sarcoma per the WHO, although T-lineage remains possible. Of 10 patients with localized disease, only 2 relapsed and 9 survive. Of 10 patients with advanced disease, 6 relapsed and 5 (50%) survive. These results suggest that localized PTCL in children and adolescents is frequently cured with modern therapy, but that advanced stage cases may require novel therapy.

Primary radiotherapy showed favorable outcome in treating extranodal nasal-type NK/T-cell lymphoma in children and adolescents

“…3,10,19,20 In contrast, analyses of small series and published case reports on pediatric patients revealed that this disease presented with a fatal clinical course, disseminated disease, and chemoresistance. [21][22][23][24][25][26] In this series, the CR rate after treatment was the lone significant prognostic factor in univariate analysis, whereas the other clinical parameters did not have any additional impact on the treatment outcome. This result can be attributed to the limited number of patients evaluated in the statistical analysis.…”

“…There are few cases reported in the literature. [21][22][23][24][25][26] In the present study, we analyzed the disease-related factors in a large series of children and adolescents with extranodal nasal-type NK/T-cell lymphoma to better characterize the clinical features, prognosis, and outcomes of this uncommon childhood malignancy.…”

Spontaneous improvement in a pediatric patient with peripheral T‐cell lymphoma

“…9 Although there are case reports on pediatric PTCL, 2,5,6 large case series are very rare, and only two have been published to date: a UK report 3 and a Children's Oncology Group (COG) study. 4 In the UK series, 25 cases were identified: 44% of children died and the 5 year survival rate was 59%. In contrast, in the 20 patients in the COG series, the 5 year survival rate was 90% in patients with localized disease and 50% in those with advanced disease.…”

Lymphomas

“…Systemic or "B" symptoms are common and a secondary hemophagocytic syndrome may be present (Jaffe 2006 ;Falini et al 1990 ). Other forms of non-anaplastic PTCL reported in children include extranodal NK/T-cell nasal type, hepatosplenic, angioblastic, angiocentric, and subcutaneous panniculitis-like PTCL (Hutchison et al 2008 ;Windsor et al 2008 ;Kobayashi et al 2010 ;Al Mahmoud et al 2012 ). The most common non-anaplastic PTCL in East Asian children is extranodal NK/T-cell nasal type (Kobayashi et al 2010 ;Hutchison et al 2008 ).…”

“…Among this boutique group of malignancies "PTCL-not otherwise specifi ed" (PTCL-NOS) appears to be the most common subtype among children in North America and the United Kingdom (Hutchison et al 2008 ;Windsor et al 2008 ;Al Mahmoud et al 2012 ). PTCL-NOS is an aggressive neoplasm.…”