Childhood Non-Hodgkin Lymphoma Treatment (PDQ®)

Incidence

Lymphoma (Hodgkin lymphoma and NHL) is the third most common childhood malignancy, and NHL accounts for approximately 7% of cancers in children younger than 20 years in the United States.[3]

The following factors affect the incidence of NHL in children and adolescents:[2,3]

• Geographic location: In the United States, about 1,200 new cases of NHL are diagnosed each year in children and adolescents younger than 20 years.[5] The incidence is approximately 13 cases per 1 million people per year.[2,3]

  In sub-Saharan Africa, the incidence of Epstein-Barr virus (EBV)–induced Burkitt lymphoma is tenfold to twentyfold higher than the incidence in the United States, resulting in a much higher incidence of NHL.[6]

• Race: The incidence of NHL is higher in White people than in Black people. Burkitt lymphoma is more common in non-Hispanic White people (3.2 cases/million person-years) than in Hispanic White people (2 cases/million person-years).[7]
• Age: Although there is no sharp age peak, childhood NHL occurs most commonly in the second decade of life.[2,3] NHL occurs infrequently in children younger than 3 years [2,3] and is very rare in infants (1% in Berlin-Frankfurt-Münster [BFM] trials from 1986 to 2002).[8]

  The incidence of NHL is increasing overall because of a slight increase in the incidence for patients aged 15 to 19 years. Conversely, the incidence of NHL in children younger than 15 years has remained constant over the past several decades.[3]

• Sex: Childhood NHL is more common in males than in females, with the exception of primary mediastinal B-cell lymphoma, in which the incidence is almost the same in males and females.[9,10] According to data from the National Childhood Cancer Registry from 2016 to 2020, the incidence of Burkitt lymphoma was 2.4 cases per 1 million children and adolescents younger than 20 years. Males have a higher incidence of Burkitt lymphoma than females (3.8 cases vs. 0.9 cases per 1 million).[3] The incidence of diffuse large B-cell lymphoma increases with age in both males and females. The incidence of lymphoblastic lymphoma remains relatively constant across ages for both males and females.[9]

The incidence and age distribution of histological types of NHL according to sex is described in Table 1.

Table 1. Incidence and Age Distribution of Specific Types of NHLa

	Incidence of NHL per Million Person-Years
	Males				Females
ALCL = anaplastic large cell lymphoma; DLBCL = diffuse large B-cell lymphoma; NHL = non-Hodgkin lymphoma.
aAdapted from Percy et al.[9]
bIndolent and aggressive histologies (more commonly seen in adult patients) are mostly found in older adolescents.
Age (y)	<5	5–9	10–14	15–19	<5	5–9	10–14	15–19
Burkitt	3.2	6	6.1	2.8	0.8	1.1	0.8	1.2
Lymphoblastic	1.6	2.2	2.8	2.2	0.9	1.0	0.7	0.9
DLBCL	0.5	1.2	2.5	6.1	0.6	0.7	1.4	4.9
Other (mostly ALCL)	2.3	3.3	4.3	7.8b	1.5	1.6	2.8	3.4b