Lymphoma in Children

Classical Hodgkin lymphoma has 4 subtypes, named for how they look under the microscope:

• Nodular sclerosing Hodgkin lymphoma
• Mixed-cellularity Hodgkin lymphoma
• Lymphocyte-rich Hodgkin lymphoma
• Lymphocyte-depleted Hodgkin lymphoma

The most common type of classical Hodgkin lymphoma in children and young adults is the nodular sclerosing type.

This type of Hodgkin lymphoma is less common in children. Only 5 to 10% of children with Hodgkin lymphoma have this type, and it is more common in children less than 10 years of age. NLPHL is treated differently than other types of Hodgkin lymphoma.

Lymphoblastic lymphoma (LBL) accounts for close to 20% of all non-Hodgkin lymphomas in children and teens in the United States. Boys are about twice as likely to develop LBL as girls.

The cancer cells of LBL are very immature lymphocytes called lymphoblasts. They are the same cells as those seen in acute lymphoblastic leukemia (ALL) in children. In fact, if more than 25% of the bone marrow is made up of lymphoblasts, the disease is classified and treated as ALL instead of lymphoma.

LBL often grows quickly with large lymph nodes in the neck and chest area.

Nodes in the chest may not be obvious by feeling or looking at a person and may need imaging tests to find. These nodes can get big enough to cause symptoms like shortness of breath, trouble lying flat, or swelling in the face and chest.

LBL can also develop in the tonsils, lymph nodes of the neck, or other lymph nodes. It can spread very quickly to the bone marrow, other lymph nodes, the surface of the brain, and/or the membranes that surround the lungs and heart.

• 3 out of 4 cases of LBL develop from T cells and are called precursor T-lymphoblastic lymphomas.
• 1 out of 4 LBLs develop from B cells and are called precursor B-lymphoblastic lymphomas. These lymphomas more often begin in lymph nodes outside the chest, particularly in the neck. They can also involve the skin and bones.

These are cancers of mature B cells. Burkitt is the most common pediatric non-Hodgkin lymphoma.

Burkitt lymphoma

Burkitt lymphoma accounts for about 40% of all childhood non-Hodgkin lymphomas in the United States. It is most often seen in boys, usually between the ages of 5 and 14 years old.

Some cases of Burkitt lymphoma are associated with infections like Epstein-Barr virus (EBV), malaria, and HIV. These infections are thought to trigger the gene changes that result in some Burkitt lymphomas.

In certain parts of Africa, Burkitt lymphoma accounts for nearly all childhood non-Hodgkin lymphomas and over half of all childhood cancers. Among children in Africa, this lymphoma usually develops in the jaw or other facial bones.

In the United States and other parts of the world, Burkitt lymphomas most often start in the abdomen (belly). Typically, a child develops a large tumor in their abdomen. This can sometimes block their bowels (intestines), causing belly pain, nausea, and vomiting.

Burkitt lymphoma can also sometimes start in the neck or tonsils, or rarely in other parts of the body.

High-grade B-cell lymphoma with 11q aberrations

This lymphoma looks like Burkitt lymphoma under the microscope but has a specific gene change that makes it different. It also shares some features with diffuse large B-cell lymphoma, described below.

These are cancers of mature B cells. Diffuse large B-cell lymphoma (DLBCL) is the most common large B-cell lymphoma in children and is more common in older children.

Diffuse large B-cell lymphoma (DLBCL)

DLBCL accounts for 15% to 20% of childhood non-Hodgkin lymphomas.

It starts in mature forms of B cells called lymphocytes, and it can grow almost anywhere in the body. It tends to happen more often in older children and teens than younger children.

Primary mediastinal B-cell lymphoma (PMBCL)

This is a related but less common type of NHL that grows as a large mass in the mediastinum (the space between the lungs). It tends to be seen most often in older teens.

Large B-cell lymphoma with IRF4 or ALK gene changes

Two additional types of large B-cell lymphoma are defined by specific gene changes. These are:

• Large B-cell lymphoma with IRF4 gene change
• Large B-cell lymphoma with ALK gene change

IRF4 gene changes have been associated with a better outlook.

Follicular lymphoma is a cancer of the B cells. It is very rare in children and adolescents, but it makes up about 30% of non-Hodgkin lymphoma in adults. Pediatric-type follicular lymphoma is more common in boys than girls.

When it happens in younger people, it is often distinctly different from follicular lymphomas in adults. Adult follicular lymphomas have a characteristic chromosome change that produces more of a specific protein called BCL-2. In children and adolescents this is rare, suggesting these cancers are different.

Children are also more likely to have cancer only in one area, where adults are more likely to have disease that has spread to other parts of the body.

Pediatric nodal marginal zone lymphoma is a rare B-cell lymphoma in children. It is much more common in boys than girls. This type of lymphoma is likely to present as swollen lymph nodes in one area (localized).

MALT lymphoma

When the marginal zone lymphoma occurs in the stomach lining, it is called MALT lymphoma. This type of marginal zone lymphoma can be related to an infection called H. pylori. When this happens, antibiotics may also be given.

This type of lymphoma makes up about 10% of all non-Hodgkin lymphomas in children and teens. It usually develops from mature T cells. It may start in lymph nodes in the neck or other areas, and it may also be found in the skin, lungs, bone, digestive tract, or other organs.

ALCL is more common in older children and teens than in younger children. 1 out of 3 cases of ALCL are localized and about 2 out of 3 have spread (disseminated) when they are diagnosed. It is uncommon for ALCL to be found in the bone marrow or central nervous system (CNS).

This is a rare mature T-cell lymphoma. Certain types of peripheral T-cell lymphomas have been seen in children with autoimmune conditions like celiac and Crohn’s disease.

Cutaneous T-cell lymphoma is a lymphoma of the skin. It is very rare in children and slightly more common in adolescents and young adults, though still rare.

The 2 most common types of cutaneous T-cell lymphomas are mycosis fungoides and lymphomatoid papulosis.

Some cases of cutaneous T-cell lymphoma can trigger hemophagocytic syndrome. This is an overactivation of the immune system that can be life-threatening if not treated quickly.

This type of lymphoma occurs in the central nervous system (brain and spinal cord) and the spinal fluid. It is rare in children and adults. Because it is rare, doctors aren’t sure if these cancers are the same in people of different ages.

Some research suggests children with CNS lymphoma may respond better to treatment than adults.

Gray zone lymphomas are a rare mix of an unclassifiable B-cell lymphoma and classical Hodgkin lymphoma. They can both be present at the time of diagnosis or found later if the lymphoma comes back (relapses) after treatment.