Mature (non‐anaplastic, non‐cutaneous) T‐/NK‐cell lymphomas in children, adolescents and young adults: state of the science

Abstract

Mature (non-anaplastic) T-cell and natural killer (NK)-lymphomas rarely occur in children or adolescents. Due to the low incidence and heterogeneity, information regarding the aetiology, physiopathology and genetics of paediatric mature (non-anaplastic) T/NK-cell lymphoma is lacking. In addition, standard treatments have not yet been established. In the absence of randomised clinical trials, anthracycline-containing regimens are usually considered as the first treatment option, but with discouraging outcomes, especially in patients with advanced disease. The implementation of autologous or allogeneic stem cell transplantation as upfront consolidation therapy or for chemotherapy-sensitive relapsed disease have resulted in improved survival for some patient subsets. The recent use of novel targeted molecular and immunotherapeutic agents has also been shown to be promising in small numbers of patients. In this context, we will review the current state of the scientific knowledge on the most common mature (non-anaplastic, non-cutaneous) T/NK-cell lymphomas occurring in children, adolescent and young adults.

First-Time Diagnosis and Ultrasound Features of Extranodal Lymphoma in Children

“…In the case of extranodal lymphatic involvement in the abdominopelvic organs, the ultrasound showed a mass echogenicity of the involved organs, which could be the stomach wall, intestinal wall, and mesentery [ 10 ], pancreas, spleen, kidneys, ovaries, and testes [ 11 ], with clear or indistinct borders and uneven internal hypoechogenicity. The involvement of the intestinal canal may be associated with intussusception [ 12 ], and the involvement of the liver parenchyma or Glison sheath may present as a cuffing sign. The involvement of extranodal lymphoma in the soft tissues of the maxillofacial region was characterized by parenchymal echogenic masses with localized bone destruction, and the involvement of subcutaneous tissue in the skin was manifested by swelling [ 13 ].…”

Durable Response in Relapsed Adolescent Peripheral T-cell Lymphoma: A Case Report and Review of the Literature

“…In 2009, pralatrexate was Food and Drug Administration (FDA) approved for relapsed or refractory PTCL in adults. 19 Pralatrexate is similar to methotrexate, an antimetabolite drug, but with a greater antitumor effect in tumor models. It was found to have good efficacy in clinical trials, either used alone or in combination with chemotherapy.…”

“…It was found to have good efficacy in clinical trials, either used alone or in combination with chemotherapy. 19 No pediatric or AYAspecific data are currently available. Romidepsin and belinostat are also both FDA approved for relapsed and refractory PTCL in the adult population.…”

Characteristics of genetic alterations of peripheral T‐cell lymphoma in childhood including identification of novel fusion genes: the Japan Children’s Cancer Group (JCCG)

“…In adults, the percentage rate of PTCL of all NHLs is relatively higher on the Asian continent than among Western countries (15–20% vs. 10–15%, respectively), and PTCL‐NOS is more frequently observed in Western than Asian countries, while NK/T‐cell lymphoma (NKTCL) and adult T‐cell leukaemia/lymphoma (ATLL) are more common types in Asia 8 . In children, anaplastic large‐cell lymphoma (ALCL) is the most common type of PTCL, accounting for ˜10% of paediatric NHLs, whereas non‐anaplastic PTCLs are rare and account for only ˜1% of NHL cases and Epstein–Barr virus (EBV)‐positive (EBV + ) T‐cell lymphoproliferative disorders (EBV‐T‐LPDs), including systemic EBV + T‐cell lymphoma of childhood (Sys‐EBV + TCL) and chronic active EBV infection of T‐ and NK‐cell type, occur predominantly in Asians and Native Americans 9,10 …”