Incidence and outcomes of rare paediatric non‐hodgkin lymphomas

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

“…Data on the incidence of other subtypes of T/NK‐lymphoma in children across the globe is more limited. In the United States (US), the incidence of mature T/NK‐cell lymphomas (excluding ALCL and cutaneous forms) varies between 0·05 and 0·34 cases/million population/year, according to subtype, among patients aged younger than 21 years (Sorge et al , ).…”

“…This subtype predominantly occurs in middle‐aged males (40–59 years) (Oshimi, ; Suzuki, ). The incidence is less frequent in children or young adults (Pillai et al , ; Sorge et al , ). In a large international database including ENKL patients from Japan, Korea, and Hong Kong ( N = 918) only about 3% (26/918 patients) were younger than 20 years, while 8·7% ( N = 80) were aged between 20 and 29 years, and 12·5% ( N = 115) were between 30 and 39 years of age (Suzuki, ).…”

“…It is estimated that hepatosplenic T‐cell lymphoma (HSTL) occurs in less than 1% of pNHL cases, with an annual incidence of 0·05 cases/million population in patients younger than 21 years (Sorge et al , ). While EATL is a disease of the elderly, HSTL occurs more often in AYA (Vose et al , ).…”

“…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 adults, this category is the most common form of PTCL with an annual incidence of 0·250 cases/100 000 population (Abouyabis et al , ). In children, PTCL‐NOS is the most common form of PTCL after ALCL, representing about 3% of all pNHL with an annual incidence of 0·34 cases/million population in the US (Sorge et al , ). In adults, PTCL‐NOS usually peaks in the 5th decade of life, while in children, the median age of presentation is 12 years (Sorge et al , ).…”