Young People with Cancer
A Handbook for Parents

National Cancer Institute
National Institutes of Health

Solid Tumors

What Is a Solid Tumor?

The word tumor does not always imply cancer. Some tumors (collections of abnormally growing cells) are benign (not cancerous). In discussing tumors that are malignant (cancerous), however, the term solid tumor is used to distinguish between a localized mass of tissue and leukemia. (Leukemia is actually a type of tumor that takes on the fluid properties of the organ it affects-the blood.)

Different kinds of solid tumors are named for the type of cells of which they are composed:

• Sarcomas
  Cancers arising from connective or supporting tissues such as bone or muscle.
  
• Carcinomas
  Cancers arising from the body's glandular cells and epithelial cells, which line body tissues.
  
• Lymphomas
  Cancers of the lymphoid organs such as the lymph nodes, spleen, and thymus, which produce and store infection-fighting cells. These cells also occur in almost all tissues of the body, and lymphomas therefore may develop in a wide variety of organs.

Kinds of Solid Tumors in Young People

Lymphomas

Lymphomas are cancers of the lymphatic tissues, which make up the body's lymphatic system. This system is a circulatory network of:

• Vessels carrying lymph (an almost colorless fluid that arises from many body tissues).
• Lymphoid organs such as the lymph nodes, spleen, and thymus that produce and store infection fighting cells.
• Certain parts of other organs such as the tonsils, stomach, small intestine, and skin.

Lymphomas have been broadly divided into Hodgkin's disease and non-Hodgkin's lymphomas, which include a number of diseases. Hodgkin's disease tends to involve peripheral lymph nodes (those near the surface of the body), where the first sign of disease may be a painless swelling in the neck, armpit, or groin. Hodgkin's disease occurs most commonly in patients in their twenties and thirties and occasionally in adolescents; it is rare in younger children.

In children, non-Hodgkin's lymphomas most frequently occur in the bowel, particularly in the region adjacent to the appendix, and in the upper midsection of the chest, a site where Hodgkin's disease may also occur. An initial sign of disease in non-Hodgkin's lymphoma may be abdominal pain or swelling, breathing difficulties and sometimes difficulty in swallowing, or swelling of the face and neck. Non-Hodgkin's lymphomas may also occur in other organs, including the liver, spleen, bone marrow, lymph nodes, central nervous system, and bones. Lymphomas can be diagnosed definitively only through a biopsy, where a piece of tumor tissue is obtained surgically and examined under a microscope. Once the diagnosis is made, many tests must be done to determine the extent of the tumor, including special x-rays, CT scans, isotope scans, and ultrasound. Blood tests are also necessary.

In the case of Hodgkin's disease, radiation therapy is highly effective for localized disease and has been the main form of treatment. However, it is believed that most lymphomas in young people are spread throughout the body, even though tumors may be detected in only one region. Because chemotherapy acts on cells throughout the body, it is the most important aspect of treatment. Surgery and radiation therapy are sometimes valuable in particular circumstances. Except in Hodgkin's disease, treatment is usually given to prevent the spread of disease to the brain and spinal column.

Brain Tumors

Brain tumors are classified and named for the type of tissue in which they develop. As a group, brain tumors are the second most common cancers of childhood. They may occur at any age, including early infancy and in adolescence, but are seen most often in children 5 to 10 years old.

Symptoms include seizures, morning headaches, vomiting, irritability, behavior problems, changes in eating or sleeping habits, lethargy, or clumsiness. Diagnosis is often difficult, because these symptoms can and frequently do indicate any number of other problems, either physical or emotional. IŁ a brain tumor is suspected, diagnostic tests usually include skull x-rays, a brain scan, CT scans, and magnetic resonance imaging (MRI).

Treatment depends on the type of brain tumor involved. For the most part, surgery, radiation, or both are used. Anticancer drugs that can be given intravenously or orally and penetrate the brain and central nervous system are also used to treat brain tumors.

Neuroblastoma

Neuroblastoma arises from very young nerve cells that, for unknown reasons, develop abnormally. More than half of these tumors occur in the adrenal glands, which are located in the abdominal area near the kidneys. Neuroblastoma is found in children only, with one-fourth of those affected showing initial symptoms during the first year of life and three-fourths before age 5.

Symptoms include a mass, listlessness, persistent diarrhea, and pain in the abdomen or elsewhere. Again, these symptoms can point to other conditions. Diagnostic tests include an intravenous pyelogram (IVP), blood tests, ultrasound, and other procedures, depending on the site of the cancer. Because most children with this particular cancer secrete a substance that can be detected in the urine, urine tests may also be performed. The diagnosis may be further established by a biopsy for examination under an electron microscope.

Surgery is performed to remove as much of the cancerous growth as possible. If some remains after surgery, radiation may be used. Chemotherapy alone or combined with radiation can also be effective in treating the remaining tumor or in preventing metastases, the spread of the disease to another site.

Wilms' Tumor

Wilms' tumor is a cancer that originates in the cells of the kidney. It occurs in children from infancy to age 15, is rare in older patients, and is very different from adult kidney cancers. It may rarely be hereditary, and about 5 percent of the cases involve both kidneys.

Parents frequently bring Wilms' tumor to the attention of the doctor after they have noticed a slight swelling or a lump in their child's abdomen. Symptoms such as blood in the urine, weakness, fever, loss of appetite, or abdominal pain may or may not be present.

Diagnosis begins with a physical examination and review of the child's medical history. An IVP is the x-ray method most often used. A special x-ray tomogram of the kidney (nephrotomography), CT scan, or other specialized diagnostic X-ray tests may also be ordered. Ultrasound pictures and other types of examination may be ordered as needed.

Wilms' tumor is one of several cancers for which treatments have been developed combining surgery, radiation therapy, and chemotherapy. The way in which these three methods will be used depends upon the child's medical history and general health and, above all, on the extent of the disease. Surgical treatment of Wilms' tumor (radical nephrectomy) involves removal of the diseased kidney and neighboring tissue and lymph nodes. Radiation therapy, for example, is not often used in children under the age of 2 when their disease is localized. When radiation therapy is used after surgery, its purpose is to guard against recurrence of the cancer where the tumor has been removed. Chemotherapy is used to treat virtually all cases of Wilms' tumor.

Retinoblastoma

Retinoblastoma is a rare cancer of the eye. It may be hereditary, and one-third of the cases involve both eyes. Retinoblastoma often can be seen by looking at the young person's eye but is usually diagnosed by an examination under general anesthesia using an ophthalmoscope, an instrument used in examining the interior of the eye. The disease tends to remain localized for long periods, but in advanced stages, it can metastasize, or spread to other parts of the body. x-rays, bone marrow examination, MRI, and a bone scan can be done to check for metastases.

If diagnosed early, it is possible to destroy the tumor with radiation therapy and preserve normal vision. If the tumor is so large that there is no hope of maintaining useful vision using radiation, the eye is removed. In cases where both eyes are involved, an attempt is made to preserve vision in both eyes through treatment with radiation. When advanced disease is found in both eyes, an attempt is made to preserve vision in at least one eye. Whenever there is any possibility of useful vision, all efforts are made to preserve it. Chemotherapy, radiation, or both may also be used to treat metastases.

Rhabdomyosarcoma

Rhabdomyosarcoma, also called rhabdosarcoma, is a type of soft tissue sarcoma arising from muscle cells. It occurs slightly more frequently in males and usually affects children between the ages of 2 and 6. Although it can occur in any muscle tissue, it is generally found in the head and neck area, the pelvis, or in the extremities.

Although rhabdomyosarcoma tends to grow and spread very rapidly, fortunately its symptoms are quite obvious compared to those of other forms of childhood cancer. A noticeable lump or swelling is present in almost all cases. Other symptoms depend on the location; if the growth is near the eyes, for example, a vision problem may develop. If the neck is involved, there may be hoarseness or difficulty in swallowing. Definite diagnosis relies on biopsy. Evidence of tumor spread is sought with x-rays, tomograms, gallium scan, bone scan, liver scan, and bone marrow examination. Other procedures, such as lymphangiography, brain scan, and spinal fluid examination, may also be done, depending on the tumor's location.

 

Traditionally, surgery has been the primary treatment, followed by intensive chemotherapy and radiation. However, if the tumor is so large that surgery presents a major risk to the patient or would result in serious disfigurement or physical impairment, then chemotherapy, radiation, or both are used to reduce the tumor's size until it can be removed more safely. In some cases, the cancer can be treated effectively with chemotherapy and radiation alone.

Osteogenic Sarcoma

Osteogenic sarcoma, also called osteosarcoma, is the most common type of bone cancer in children. It arises in the ends of the bones. The bones most frequently involved are the large bones of the upper arm (humerus) and the leg (femur and tibia). Osteogenic sarcoma usually occurs between the ages of 10 and 25 and is more common among males than females.

Young people with this type of cancer generally complain of pain and swelling, which they sometimes blame on an injury. Diagnosis can be difficult, because the disease is easily confused with local infection, effects of injury, glandular deficiencies, arthritis, vitamin deficiencies, and benign tumors. Although osteogenic sarcoma may be suspected by the way the bone looks on x-rays, diagnosis can be confirmed only by biopsy. Because the disease commonly spreads (metastasizes) to other parts of the body, especially the lungs, chest x-rays, lung tomograms, CT scans of the chest, and an x-ray skeletal survey or bone scan may also be done before treatment.

Surgery (amputation or limb-sparing) is the primary method of treatment, followed by a course of chemotherapy using one or more anticancer drugs. A prosthesis (artificial limb) and physical rehabilitation may be important parts of therapy.

Ewing's Sarcoma

Ewing's sarcoma differs from osteosarcoma in that it affects a different part of the bone-the bone shaft- and tends to be found in bones other than the long bones of the arm and leg, such as the ribs. Like osteogenic sarcoma, it usually occurs between the ages of 10 and 25, is seen more often in males, and frequently spreads to other bones and the lungs.

Young people with this type of cancer usually have more general signs-fever, chills, and weakness-than are present in osteogenic sarcoma. Because the symptoms can point to other conditions, definitive diagnosis depends on biopsy. A bone survey, bone scans, chest x-rays, lung tomograms, liver scans, and brain scans may be done as well to look for evidence of metastases.

Treatment involves use of a combination of intensive radiation therapy and chemotherapy.

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