Non-Hodgkin lymphoma


What is childhood non-Hodgkin lymphoma?

Childhood non-Hodgkin lymphoma is a disease in which malignant (cancer) cells form in the lymph system.Because lymph tissue is found throughout the body, childhood non-Hodgkin lymphoma can begin in almost any part of the body. Cancer can spread to the liver and many other organs and tissues. Non-Hodgkin lymphoma can occur in both adults and children. Treatment for children is different than treatment for adults.

What are the types of childhood non-Hodgkin lymphoma?

The specific type of lymphoma is determined by how the cells look under a microscope. The 4 major types of childhood non-Hodgkin lymphoma are:

  • B-cell non-Hodgkin lymphoma (Burkitt's lymphoma and Burkitt-like lymphoma) and Burkitt leukemia
  • Diffuse large B-cell lymphoma
  • Lymphoblastic lymphoma
  • Anaplastic large cell lymphoma.

What are the symptoms of childhood non-Hodgkin lymphoma?

These and other symptoms may be caused by childhood non-Hodgkin lymphoma. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

  • Shortness of breath
  • Trouble breathing
  • Wheezing
  • High-pitched breathing sounds
  • Swelling of the head or neck
  • Trouble swallowing
  • Painless swelling of the lymph nodes in the neck, underarm, stomach, or groin
  • Fever for no known reason
  • Weight loss for no known reason
  • Night sweats

What is the prognosis for patients diagnosed with childhood non-Hodgkin lymphoma?

The prognosis (chance of recovery) and treatment options depend on:

  • The type of lymphoma
  • The stage of the cancer
  • The number of places outside of the lymph nodes to which the cancer has spread
  • Whether the lymphoma has spread to the central nervous system (brain and spinal cord)
  • Whether there are certain changes in the chromosomes
  • The type of initial treatment
  • The patient’s general health.

What are the stages of childhood non-Hodgkin lymphoma?

Stage I. Cancer is found in one group of lymph nodes or one area outside the lymph nodes, but no cancer is found in the abdomen or mediastinum (area between the lungs).

Stage II. Cancer is found in one area outside the lymph nodes and in nearby lymph nodes (a); or in two or more areas above (b) or below (c) the diaphragm; or cancer started in the stomach, appendix, or intestines (d) and can be removed by surgery.

Stage III. Cancer is found in at least one area above and below the diaphragm (a); or cancer started in the chest (b); or cancer started in the abdomen and spread throughout the abdomen (c); or in the area around the spine (not shown).

Stage IV. Cancer is found in the bone marrow, brain, or cerebrospinal fluid (CSF). Cancer may also be found in other parts of the body. Treatment for childhood non-Hodgkin lymphoma is based on whether the cancer is low-stage or high-stage. Low-stage lymphoma has not spread beyond the area in which it began. High-stage lymphoma has spread beyond the area in which it began. Stage I and stage II are usually considered low-stage. Stage III and stage IV are usually considered high-stage.

Recurrent childhood non-Hodgkin lymphoma is cancer that has recurred (come back) after it has been treated. Childhood non-Hodgkin lymphoma may come back in the lymph system or in other parts of the body.

What are the treatments for childhood non-Hodgkin lymphoma?

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas. Intrathecal chemotherapy may be used to treat childhood non-Hodgkin lymphoma that has spread, or may spread, to the brain. When used to prevent spread to the brain, it is called CNS prophylaxis. The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Intrathecal chemotherapy. Anticancer drugs are injected into the intrathecal space, which is the space that holds the cerebrospinal fluid (CSF, shown in blue). There are two different ways to do this. One way, shown in the top part of the figure, is to inject the drugs into an Ommaya reservoir (a dome-shaped container that is placed under the scalp during surgery; it holds the drugs as they flow through a small tube into the brain). The other way, shown in the bottom part of the figure, is to inject the drugs directly into the CSF in the lower part of the spinal column, after a small area on the lower back is numbed.

Radiation therapy is a cancer treatment that uses high energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. When radiation therapy is used to prevent spread to the brain, it is called CNS prophylaxis. The way the radiation therapy is given depends on the type and stage of the cancer being treated.

High-dose chemotherapy with stem cell transplant. This treatment is a way of giving high doses of chemotherapy and then replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the bone marrow or blood of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body’s blood cells.

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibodies and tyrosine kinase inhibitors are two types of targeted therapy being studied in the treatment of childhood non-Hodgkin lymphoma.

Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells.

Tyrosine kinase inhibitors (TKIs) block signals that tumors need to grow. Some TKIs also keep tumors from growing by preventing the growth of new blood vessels to the tumors. New types of treatment are being tested in clinical trials.