About this Cancer

Lymphoma is a cancer of the body’s lymphatic system. The lymphatic system helps the body fight infection and is composed of many glands located throughout the body; in the neck, armpits, chest, abdomen, and groin. Tiny lymph vessels connect the glands. The spleen, which breaks down old blood cells, and tonsils are also part of the lymphatic system.

Lymphomas are typically classified as a Hodgkins’s Disease or Hodgkin’s lymphoma or non-Hodgkin’s lymphoma. Hodgkin’s disease is a rapidly growing cancer named for the doctor who first described it in 1832, Dr. Thomas Hodgkin, and characterized by the presence of a specific type of cancer cell (Reed-Sternberg cells). Lymphomas that do not have the Hodgkin’s disease characteristic cell are called non-Hodgkin’s lymphomas. Hodgkin’s disease is the most common form of lymphoma seen in children, with 10 to 15 percent of cases diagnosed in children under 16.  On the other hand, just five percent of all non-Hodgkin’s lymphomas occur in children.

Hodgkin’s Disease

Risk Factors

The exact cause of Hodgkin’s disease is not known. A link between viral infection with the Epstein-Barr (EBV) virus and the development of Hodgkin’s disease in certain people has been made and continues to be studied.

Boys are more likely than girls to develop lymphoma of any type.

Cancer Symptoms

The first sign of Hodgkin's disease is usually a painless swelling of one lymph gland or a group of lymph glands, that does not go away. The lymph glands most commonly affected are in the neck (usually on one side only), in a small area above the collarbone, in the armpit, or groin.  Sometimes glands in the chest are affected and can cause a persistent cough or shortness of breath. Occasionally, children with Hodgkin's disease may have a fever, itching, irritation of the skin, weight loss, or night sweats.

Diagnostic Tests

To confirm a diagnosis of Hodgkin’s disease, the swollen lymph gland, or a portion of the swollen gland, must be removed through a surgical biopsy. The lymph gland is examined under a microscope by a pathologist to determine if cancer cells are present. Additional tests, including X-rays, CT (computerized tomography) scans, and blood tests are done to determine how extensively the disease has spread through the lymph system. This is referred to as "staging".

If the doctors suspect the Hodgkin’s disease has spread to the bone marrow, the spongy tissue inside large bones, they may order a bone marrow biopsy.  In a bone marrow biopsy, a needle is used to extract a small portion of bone marrow from the hip bone so that it may be examined for the presence of cancer cells.

In the past, abdominal surgery, called a staging laparotomy, was needed to biopsy the liver and remove the spleen to see if it was affected by the lymphoma. However, because other tests are now quite good at detecting the spread of Hodgkin's disease, this surgery is unnecessary most of the time.

Other tests the doctor may order are a gallium (the radioactive intake of gallium in the lymph system) scan to indicate swelling and, ultimately, disease.  Currently, PET (positron emission topography) is being evaluated to see if it will help detect any lymphoma.

Treatment

Treatment primarily involves oral and intravenous treatment with anticancer drugs known as chemotherapy. As the lymph system is located throughout the body, chemotherapy is systemic as well, traveling through the bloodstream and delivering anticancer drugs to the entire body. Patients may also receive radiation therapy, targeted X-rays that destroy specific tumors or cancers. 

A staging evaluation is necessary to determine the treatment plan. Patients having Stages I and II are usually referred to as having limited disease, while patients with Stages III and IV are considered to have extensive disease:

• Stage I indicates one lymph node region is involved (for example, the right neck)
• Stage II indicates involvement of two lymph nodes on the same side of the diaphragm (for example, both sides of the neck)
• Stage III indicates lymph node involvement on both sides of the diaphragm (for example, groin and armpit)
• Stage IV involves the spread of the disease outside the lymph nodes (for example, to the bone marrow, lungs, or liver)

After treatment with chemotherapy some children, particularly boys, may become infertile. Parents of teenage boys should be aware of the option of sperm banking. In this situation sperm can be stored for possible use in later years.  Current chemotherapy is being evaluated to decrease the long-term effects of therapy.

There is also a small risk of developing a different cancer later in life as a result of treatment for Hodgkin's disease.

Most of the time, Hodgkin’s disease can be cured with chemotherapy and/or radiation therapy.  If the cancer does not respond to standard treatment or has spread to the bone marrow, a bone marrow or stem cell transplant can be done.  In this treatment, the diseased bone marrow is destroyed using high doses of chemotherapy.  Healthy, donor (allogeneic) stems cells or bone marrow, or self-donated (autologous), stem cells or bone marrow are given to the patient intravenously, and travel to the bone to repopulate the bone marrow.

Non-Hodgkin’s Lymphoma

About this Cancer

There are many types of non-Hodgkin's lymphoma, characterized by which type of cells in the body are cancerous, and how they present themselves.  Childhood non-Hodgkin’s lymphomas fall into three broad categories: lymphoblastic lymphoma, small non-cleaved cell lymphoma (Burkitt's and non-Burkitt's) and large cell lymphoma.

Risk Factors

What causes non-Hodgkin's lymphoma is still unknown. The incidence has continued to increase over the years. The current thinking is that there is probably a genetic factor that lays the groundwork for the cancer, which is then triggered by other influences, such as environmental factors. Non-Hodgkin’s lymphoma is not contagious, and the patient does not pose a risk to others in any way.

Boys are more likely than girls to develop lymphoma of any type.

Cancer Symptoms

Non-Hodgkin’s lymphoma can be very difficult to detect. A parent, loved one, or the child may notice something wrong. There are some symptoms for non-Hodgkin's lymphoma, but they are not specific. Often, a lymph node swells, especially in the upper body area. Other times the child may feel a lack of energy. More serious symptoms can include weight loss, fever, night sweats, or unexplained itching. Unlike non-Hodgkin's lymphoma that occurs in adults, the form seen in children may grow very rapidly.

For children with small non-cleaved cell lymphoma, the signs and symptoms usually depend on the site affected by the cancer.   This malignancy grows very rapidly and a child who appeared in good health four to six weeks ago may now be ill.  The child may have a large abdominal mass with fluid buildup, and can experience pain and vomiting.

Diagnostic Tests

To confirm a diagnosis of non-Hodgkin’s lymphoma, a lymph tissue sample must be removed through a surgical biopsy. The tissue is examined under a microscope by a pathologist to determine if cancer cells are present. Additional tests, including X-rays, CT (computerized tomography) scans, and blood tests are done to determine how extensively the disease has spread.

A bone marrow biopsy will be ordered to see if the non-Hodgkin's lymphoma has spread to the bone marrow, the spongy tissue inside large bones.  In a bone marrow biopsy, a needle is used to extract a small portion of bone marrow from the hip bone so that it may be examined for the presence of cancer cells.

Other tests  may include a gallium (the radioactive intake of gallium in the lymph system) or PET (positron emission tomography) scan.

The biopsy will determine the specific type of non-Hodgkin’s lymphoma the patient has:  lymphoblastic lymphoma, small non-cleaved cell lymphoma (Burkitt’s and non-Burkitt’s), or large cell lymphoma.

Diagnostic tests also are used to "stage" the lymphoma.  Staging characterizes how extensively the lymphoma has spread throughout the body:

Stage I: Cancer is found in only one area outside of the abdomen or chest.

Stage II: Any of the following mean the disease is Stage II:

•          Cancer is found in only one area and in the lymph nodes around it.
•          Cancer is found in two or more lymph nodes or other areas on the same side of the diaphragm (the thin muscle under the lungs that helps one breathe).
•          Cancer is found to have started in the digestive tract. The lymph nodes in the area may or may not have cancer.

Stage III: Any of the following mean the disease is Stage III:

• -         Cancer is found in the lymph node areas on both sides of the diaphragm.
• -         Cancer is found to have started in the chest.
• -         Cancer is found in many places in the abdomen.
• -         Cancer is found in the area around the spine, around the outermost covering of the brain, or on the outermost covering of the brain (these tumors are called epidermal tumors).

Stage IV - Cancer has spread to the bone marrow or to the brain, its inner coverings, or the spinal cord.

Recurrent: Recurrent disease means that the cancer has come back after it has been treated. It may come back in the area where it first started, or in another part of the body.

Treatment

Chemotherapy (oral and/or intravenous treatment with anticancer drugs) is the most common therapy for non-Hodgkin’s lymphoma.  Some patients may require over two years of chemotherapy.

Lymphoblastic Lymphoma

Lymphoblastic lymphoma accounts for approximately 30 percent of childhood lymphomas. With intensive chemotherapy, long-term, disease-free survival may be attained, and the childhood complete remission rate is as high as 96 percent for certain types of lymphoblastic lymphoma.

A majority of patients have tumors above the diaphragm; abdominal involvement is uncommon.

Combined chemotherapy (oral and intravenous treatment with anticancer drugs) is standard treatment, with the specific type dependent on the stage of the disease.  Ara-C and methotrexate may also be injected into the cerebrospinal fluid, through a spinal tap, to combat central nervous system (CNS) disease, or, as a preventative measure so it does not spread to the central nervous system.

Small Non-Cleaved Cell Lymphoma

Small non-cleaved cell lymphoma (SNCL) accounts for approximately 40-50 percent of all childhood lymphomas. SNCL is an aggressive B-cell lymphoma that appears in one of three varieties:

• Endemic Burkitt's lymphoma: a childhood lymphoma (5 to 10 year olds) and is prevalent in equatorial Africa and intimately associated with both Epstein-Barr virus (EBV) infection and a characteristic translocation of the c-myc gene (a gene involved in cellular proliferation).
• Sporadic Burkitt's lymphoma: a lymphoma prevalent worldwide affecting slightly older children. It is also associated with c-myc changes, but less so with EBV infection.
• Non-Burkitt's lymphoma: a rather different disease affecting an older population and not notably associated with the c-myc gene or EBV infection. The BCL-2 gene is rearranged in 33 percent of patients.

Combined chemotherapy (oral and intravenous treatment with anticancer drugs) is standard treatment with the specific type dependent on the stage of the disease. 

Large Cell Lymphoma

Large cell immunoblastic lymphoma is an aggressive T-cell or B-cell lymphoma with a rapidly growing mass at or around the lymph nodes. There may be brain, bone, skin, or gastrointestinal tract involvement; approximately 12 percent of patients have bone marrow involvement.

This type of lymphoma is usually very responsive to chemotherapy (treatment with anticancer drugs).