 Lymphoma of Bone
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Lymphoma is a type of cancer that originates in the lymphatic system, part of the body's immune system that fights disease and infections. The lymphatic system includes:
- lymph fluid - fluid containing lymphocyte cells.
- lymph vessels - thin tubes that carry lymph fluid throughout the body.
- lymphocytes - specific white blood cells that fight infections and disease. The two main types of lymphocytes are B-cells and T-cells. Normal B-cells mature into plasma cells and produce immunoglobulins (also called antibodies) that help protect the body from bacteria. Normal T-cells serve in several roles to help protect the body. They recognize and destroy virus-infected cells, and they help attract other white blood cells that kill infected cells. There are several types and different stages of maturation for these cells.
- lymph nodes - bean-shaped organs, found in the underarm, groin, neck, chest, and abdomen, that act as filters for the lymph fluid as it circulates through the body
- spleen - a small organ that produces lymphocytes, stores healthy blood cells, and filters out damaged blood cells, bacteria and cell waste
- thymus - a small gland, located in front of the heart, that plays a vital role in the development of T-cells while a child is still in the womb. Although the size of the gland starts to decrease after young adulthood, it continues to serve as part of the immune system throughout life.
- bone marrow/stem cells - the bone marrow contains stem cells, cells that develop into red blood cells, platelets (clotting cells) and infection-fighting white blood cells, including lymphocytes. Stem cells are also found circulating throughout the body in the blood stream.
While most lymphomas originate in lymph nodes, lymphoma of the bone, which is considered a non-Hodgkin's form of the disease, arises from the bone, where lymphocytes are produced. Lymphoma of the bone causes the lymphocytes to abnormally reproduce, eventually causing tumors to grow in the bone. This type of lymphoma is considered a widespread disease, because usually multiple sites in the body are involved as primary sites. In some children however, the tumor may arise in bone only (primary lymphoma of bone). Cancerous cells can also spread to other organs and tissues in the body.
Non-Hodgkin's lymphoma is the third most common childhood cancer. It occurs most often in children between the ages of 7 and 11, but can occur at any age from infancy to adulthood. Non-Hodgkin's lymphoma affects males almost three times more often than females, and is more common among Caucasian children than among African-American children and children of other races. Staging and classification of non-Hodgkin's lymphoma is based on the extent of the disease and the specific cells involved.
In addition to lymphoma of bone, there are several types of non-Hodgkin's lymphomas, classified by physicians based on the size and shape of the lymphoma cells under a microscope, and how the cells grow within the lymph nodes and beyond.
For more information on other types of lymphoma, see Burkitt's lymphoma, large cell lymphoma, lymphoblastic lymphoma, or cutaneous or skin lymphomas.
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The specific cause of non-Hodgkin's lymphoma is unclear. It is possible that genetics and exposure to viral infections may increase the risk for developing this malignancy. Non-Hodgkin's lymphoma has also been linked to chemotherapy and radiation therapy. Non-Hodgkin's may be a second malignancy as a result of the treatment for certain cancers. There has been much investigation into the association of the Epstein-Barr virus (EBV) that causes the mononucleosis infection; as well as the human immunodeficiency virus (HIV), which causes acquired immune deficiency syndrome (AIDS). Both of these infectious viruses have been linked to the development of Burkitt's lymphoma, in particular.
There has been much investigation into the association of the Epstein-Barr virus (EBV) that causes the mononucleosis infection; as well as the human immunodeficiency virus (HIV), which causes acquired immune deficiency syndrome (AIDS). Both of these infectious viruses have been linked to the development of Burkitt's lymphoma. The majority of Burkitt's lymphoma cases result from a chromosome rearrangement between chromosome #8 and #14, which causes genes to change positions, and function differently, promoting uncontrolled cell growth.
Other chromosome rearrangements have been seen in non-Hodgkin's lymphoma (all types) that are also thought to promote excessive cell growth. Children and adults with other hereditary abnormalities have an increased risk of developing non-Hodgkin's lymphoma, including patients with ataxia telangiectasia, X-linked lymphoproliferative disease, or the Wiskott-Aldrich syndrome.
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Staging is the process of determining whether cancer has spread and, if so, how far. There are various staging symptoms that are used for non-Hodgkin's lymphoma. Always consult your child's physician for information on staging. One method of staging non-Hodgkin's lymphoma is the following:
- stage I - involves the tumor at one site, either nodal or elsewhere in the body.
- stage II - involves the tumor at two or more sites on the same side of the body.
- stage III - involves tumors in any number that occur on both sides of the body, but does not involve bone marrow or the central nervous system.
- stage IV - is any stage of tumor that also has bone marrow and/or central nervous system involvement. Stage IV is also subdivided depending on the amount of blasts (cancer cells) present in the bone marrow.
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Most children have stage III or IV disease at the time of diagnosis because of the sudden onset of symptoms. The disease can progress quickly from a few days to a few weeks. A child can go from otherwise healthy to having multi-system involvement in a short time period.
Some children with non-Hodgkin's lymphoma have symptoms of an abdominal mass and have complaints of abdominal pain, fever, constipation, and decreased appetite - due to the pressure and obstruction a large tumor in this area can cause. Some children with non-Hodgkin's lymphoma have symptoms of a mass in their chest and have complaints of respiratory problems, pain with deep breaths (dyspnea), cough, and/or wheezing.
Because of the rapid onset of this malignancy, any respiratory symptoms can quickly worsen, causing a life-threatening emergency.
The following are the most common symptoms of non-Hodgkin's lymphoma. However, each child may experience the symptoms differently. Symptoms may include:
- painless swelling of the lymph nodes in neck, chest, abdomen, underarm, or groin
- fever
- sore throat
- fullness in groin area from node involvement
- bone and joint pain
- night sweats
- tiring easily (fatigue)
- weight loss/decreased appetite
- itching of the skin
- recurring infections
The symptoms of non-Hodgkin's lymphoma may resemble other blood disorders or medical problems. Always consult your child's physician for a diagnosis.
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In addition to a complete medical history and physical examination, diagnostic procedures for non-Hodgkin's lymphoma may include:
- blood and urine tests
- x-rays of the chest - uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film
- computerized tomography scan of the abdomen, chest, and pelvis (also called a CT or CAT scan) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.
- lymph node biopsy - a sample of tissue is removed from the lymph node and examined under a microscope
- lymphangiogram - an imaging study that can detect cancer cells or abnormalities in the lymphatic system and structures. It involves a dye being injected into the lymph system.
- bone marrow aspiration/biopsy (to detect cancer cells) - a procedure that involves a small amount of bone marrow fluid and tissue to be taken, usually from part of the hip bones, to further examine the number, size, and maturity of blood cells and/or abnormal cells
- lumbar puncture (to evaluate central nervous system disease for cancer cells) - a special needle is placed into the lower back, into the spinal canal. This is the area around the spinal cord. A small amount of cerebral spinal fluid (CSF) can be removed and sent for testing. CSF is the fluid that bathes the brain and spinal cord.
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Specific treatment for lymphoma of bone will be determined by your child's physician based on:
- your child's age, overall health, and medical history
- the extent of the disease
- your child's tolerance for specific medications, procedures, or therapies
- the expectations for the course of the disease
- your opinion or preference
Treatment for lymphoma of the bone is handled is similar fashion to treatment of other non-Hodgkin's lymphomas that originate in lymph nodes. It is treated differently than other types of malignant bone sarcomas. Treatment may include (alone or in combination):
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- chemotherapy - a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce. Different groups of drugs work in different ways to fight cancer cells and shrink tumors. Chemotherapy may be used alone for some types of cancer or in conjunction with other therapy such as radiation. Often, a combination of chemotherapy drugs is used to fight a specific cancer. Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat. While chemotherapy can be quite effective in treating certain cancers, the agents do not differentiate normal healthy cells from cancer cells. Because of this, there can be many adverse side effects during treatment. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring, if possible. Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Chemotherapy can be given:
- intravenously (directly to the bloodstream; also called IV)
- intrathecally - chemotherapy given directly into the spinal column with a needle
- surgery - surgery usually plays a limited role in the treatment of lymphoma. In some instances, a child may need to have a tumor removed. This may be the case if, for example, the tumor originates in the mediastinum region, where it may compress the airway and/or the heart and major vessels. Surgery in this area may entail considerable risk, and must be carefully orchestrated between the surgeon, oncologist and anesthesiologist. In rare instances a lymphoma may weaken one or more bones. In addition to radiation, a special plate or rod may be necessary to prevent or treat a fracture of the involved bone.
- radiation therapy - using high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors, if there is a bulky tumor involved.
- stem cell transplant - a treatment involving stem cells, a specific type of cell from which all blood cells develop. Stem cells develop into red blood cells to carry oxygen, white blood cells to fight disease and infection, and platelets to aid in blood clotting. Stem cells are found primarily in the bone marrow, but some also circulate in the blood stream.Sometimes, stem cells are collected from patients themselves prior to intensive treatment and used to supplement the recovery after a particularly aggressive course of treatment. Through this procedure, stem cells are taken from the patient, treated in a laboratory to kill any remaining cancer cells, and kept in frozen storage for later use. After chemotherapy and/or radiation therapy, the saved stem cells are thawed and given back to the patient to grow new bone marrow and other stem cells. The use of a patient's own cells is referred to as autologous transplantation.
- antibiotics - to prevent or treat infections
- supportive care - for side effects of treatment
- long-term follow up care - to determine response to treatment, detect recurrent disease, and manage late effects of treatment
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Prognosis greatly depends on:
- the extent of the disease
- the presence or absence of metastasis
- the response to therapy
- age and overall health of the child
- your child's tolerance of specific medications, procedures, or therapies
- new developments in treatment
As with any cancer, prognosis and long-term survival can vary greatly from child to child. Every child is unique and treatment and prognosis is structured around the child. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for the child diagnosed with non-Hodgkin's lymphoma. Side effects of treatment, as well as second malignancies, can occur in survivors of non-Hodgkin's lymphoma. New methods are continually being discovered to improve treatment and to decrease side effects.
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The Dana-Farber/Children Hospital program is part of the Children's Oncology Group, a national group of pediatric oncologists whose work includes investigational treatments for newly diagnosed patients with non-Hodgkin's lymphoma and those who experience relapse. Studies also seek to improve the supportive care of patients undergoing treatment.
One area of treatment under investigation is biological therapy or immunotherapy, the use of drugs that seem to slow the growth of cancer cells while stimulating and strengthening the body's own immune system. Improvements are also occurring in the area of stem cell transplant, including ways to improve pre-transplant treatment, broaden the range of compatible donors, and developing more effective ways of removing cancerous cells from patient's own bone marrow.
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