Unusual Cancers of Childhood Treatment (PDQ®)–Patient Version
Unusual Cancers of the Chest
Breast Cancer
Breast cancer is a disease in which malignant (cancer) cells form in the tissues of the breast. Breast cancer can occur in the breast tissue of both male and female children.
Breast cancer is the most common cancer among females aged 15 to 39 years; however, less than 5% of breast cancers occur in females in this age group. Breast cancer in this age group is more aggressive and more difficult to treat than in older women. Treatments for younger and older women are similar. Younger patients with breast cancer may have genetic counseling (a discussion with a trained professional about inherited diseases) and testing for family cancer syndromes. Also, the possible effects of treatment on fertilityshould be considered.
Most breast tumors in children are fibroadenomas, which are benign (not cancer). Rarely, these tumors become large phyllodes tumors (cancer) and begin to grow quickly. If a benign tumor begins to grow quickly, a fine-needle aspiration (FNA) biopsy or an excisional biopsy will be done. The tissues removed during the biopsy will be viewed under a microscope by a pathologist to check for signs of cancer.
Risk Factors, Signs, and Diagnostic and Staging Tests
The risk of breast cancer is increased by the following:
- Having a personal history of a type of cancer that may spread to the breast, such as leukemia, rhabdomyosarcoma, soft tissue sarcoma, or lymphoma.
- Past treatment for another cancer, such as Hodgkin lymphoma, with radiation therapyto the breast or chest.
Other risk factors for breast cancer include the following:
- A family history of breast cancer in a mother, father, sister, or brother.
- Inherited changes in the BRCA1 or BRCA2 gene or in other genes that increase the risk of breast cancer.
Breast cancer may cause any of the following signs. Check with your child’s doctor if your child has any of the following:
- A lump or thickening in or near the breast or in the underarm area.
- A change in the size or shape of the breast.
- A dimple or puckering in the skin of the breast.
- A nipple turned inward into the breast.
- Fluid, other than breast milk, from the nipples, including blood.
- Scaly, red, or swollen skin on the breast, nipple, or areola (the dark area of skin that is around the nipple).
- Dimples in the breast that look like the skin of an orange, called peau d’orange.
Other conditions that are not breast cancer may cause these same signs.
- Physical exam and health history.
- MRI.
- Ultrasound.
- PET scan.
- Blood chemistry studies.
- X-ray of the chest.
- Biopsy.
See the General Information section for a description of these tests and procedures.
Other tests that may be used to diagnose breast cancer include the following:
- Clinical breast exam (CBE): An exam of the breast by a doctor or other health professional. The doctor will carefully feel the breast and under the arm for lumps or anything else that seems unusual.
- Mammogram: An x-ray of the breast. When treatment for another cancer included radiation therapy to the breast or chest, it is important to have a mammogram and MRI of the breast to check for breast cancer. These should be done beginning at age 25, or 10 years after finishing radiation therapy, whichever is later.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of benign breast tumors in children may include the following:
- Watchful waiting. These tumors may disappear without treatment.
- Surgery to remove the tumor.
Treatment of breast cancer in children may include the following:
- Surgery to remove the tumor, but not the whole breast. Radiation therapy may also be given.
Treatment of recurrent breast cancer in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
See the PDQ summary Breast Cancer Treatment for more information on the treatment of adolescents and young adults with breast cancer.
Lung Cancer
Lung cancer is a disease in which malignant cancer cells form in the tissue of the lung. The lungs are a pair of cone-shaped breathing organs in the chest. The lungs bring oxygen into the body as you breathe in. They release carbon dioxide, a waste product of the body’s cells, as you breathe out. Each lung has sections called lobes. The left lung has two lobes. The right lung is slightly larger and has three lobes. Two tubes called bronchi lead from the trachea (windpipe) to the right and left lungs. Tiny air sacs called alveoli and small tubes called bronchioles make up the inside of the lungs.
In children, most lung or airway tumors are malignant (cancer). The following are the most common primary lung or airway tumors:
- Tracheobronchial tumors.
- Pleuropulmonary blastoma.
This summary is not about cancer that has spread to the lungs from another part of the body.
Tracheobronchial Tumors
Tracheobronchial tumors begin in the inside lining of the trachea or bronchi. Most tracheobronchial tumors in children are benign and occur in the trachea or large bronchi (large airways of the lung). Sometimes, a slow-growing tracheobronchial tumor, such asinflammatory myofibroblastic tumor, becomes cancer that may spread to other parts of the body.
Signs and Symptoms
Tracheobronchial tumors may cause any of the following signs and symptoms. Check with your child's doctor if your child has any of the following:
- Dry cough.
- Wheezing.
- Trouble breathing.
- Spitting up blood from the airways or lung.
- Frequent infections in the lung, such as pneumonia.
- Feeling very tired.
- Loss of appetite or weight loss for no known reason.
Other conditions that are not tracheobronchial tumors may cause these same signs and symptoms. For example, symptoms of tracheobronchial tumors are a lot like the symptoms of asthma, which can make it hard to diagnose the tumor.
Diagnostic and Staging Tests
Tests to diagnose and stage tracheobronchial tumors may include the following:
- Physical exam and health history.
- X-ray of the chest.
- CT scan.
See the General Information section for a description of these tests and procedures.
Other tests used to diagnose tracheobronchial tumors include the following:
- Bronchography: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. A contrast dye is put through the bronchoscope to make the larynx, trachea, and airways show up more clearly on x-ray film.
- Octreotide scan: A type of radionuclide scan used to find tracheobronchial tumors or cancer that has spread to the lymph nodes. A very small amount of radioactiveoctreotide (a hormone that attaches to carcinoid tumors) is injected into a vein and travels through the bloodstream. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body.
Prognosis
The prognosis (chance of recovery) for children with tracheobronchial cancer is very good, unless the child has rhabdomyosarcoma.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
The treatment of tracheobronchial tumors depends on the type of cell the cancer formed from. Treatment of tracheobronchial tumors in children may include the following:
- Surgery to remove the tumor. Sometimes a type of surgery called a sleeve resection is used. The lymph nodes and vessels where cancer has spread are also removed.
- Targeted therapy (crizotinib), for inflammatory myofibroblastic tumors that form in the trachea or bronchi.
- Chemotherapy and radiation therapy, for rhabdomyosarcoma that forms in the trachea or bronchi.
Treatment of recurrent tracheobronchial tumors in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
See the Neuroendocrine Tumors (Carcinoid Tumors) section of this summary for more information.
Pleuropulmonary Blastoma
Pleuropulmonary blastomas (PPBs) form in the tissue of the lung and pleura (tissue that covers the lungs and lines the inside of the chest). They can also form in the organsbetween the lungs including the heart, aorta, and pulmonary artery, or in the diaphragm(the main breathing muscle below the lungs). In most cases, PPBs are linked to a certain change in the DICER1 gene.
There are three types of PPB:
- Type I tumors are cyst-like tumors in the lung. They are most common in children aged 2 years and younger and have a good chance of recovery. Type Ir tumors are Type I tumors that have regressed (gotten smaller) or have not grown or spread. After treatment, a Type I tumor may recur as a Type II or III tumor.
- Type II tumors are cyst-like with some solid parts. These tumors sometimes spread to the brain or other parts of the body.
- Type III tumors are solid tumors. These tumors often spread to the brain or other parts of the body.
Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests
The risk of PPB is increased by the following:
- Having a certain change in the DICER1 gene.
- Having a family history of DICER1 syndrome.
PPB may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:
- A cough that doesn’t go away.
- Trouble breathing.
- Fever.
- Lung infections, such as pneumonia.
- Pain in the chest or abdomen.
- Loss of appetite.
- Weight loss for no known reason.
Other conditions that are not PPB may cause these same signs and symptoms.
- Physical exam and health history.
- X-ray of the chest.
- CT scan of the chest and abdomen.
- PET scan.
- MRI of the head.
- Bone scan.
See the General Information section for a description of these tests and procedures.
Other tests used to diagnose PPB include the following:
- Bronchoscopy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
- Thoracoscopy: A surgical procedure to look at the organs inside the chest to check for abnormal areas. An incision (cut) is made between two ribs, and a thoracoscope is inserted into the chest. A thoracoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. In some cases, this procedure is used to remove part of the esophagus or lung. If the thoracoscope cannot reach certain tissues, organs, or lymph nodes, a thoracotomy may be done. In this procedure, a larger incision is made between the ribs and the chest is opened.
PPBs may spread or recur (come back) even after being removed by surgery.
Prognosis
The prognosis (chance of recovery) depends on the following:
- The type of pleuropulmonary blastoma.
- Whether the tumor has spread to other parts of the body at the time of diagnosis.
- Whether the tumor was completely removed by surgery.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of pleuropulmonary blastoma in children includes the following:
- Surgery to remove the whole lobe of the lung the tumor is in, with or without chemotherapy.
Treatment of recurrent pleuropulmonary blastoma in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
Esophageal Tumors
Esophageal tumors may be benign (not cancer) or malignant (cancer). Esophageal canceris a disease in which malignant cells form in the tissues of the esophagus. The esophagus is the hollow, muscular tube that moves food and liquid from the throat to the stomach. Most esophageal tumors in children begin in the thin, flat cells that line the inside of the esophagus.
Risk Factors and Signs and Symptoms
The risk of esophageal cancer is increased by the following:
- Swallowing chemicals, which may burn the esophagus.
- Having gastroesophageal reflux.
- Having Barrett esophagus.
Esophageal cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:
Other conditions that are not esophageal cancer may cause these same signs and symptoms.
Diagnostic and Staging Tests
- Physical exam and health history.
- X-ray of the chest.
- CT scan.
- PET scan.
- MRI.
- Ultrasound.
See the General Information section for a description of these tests and procedures.
Other tests used to diagnose esophageal cancer include the following:
- Barium swallow: A series of x-rays of the esophagus and stomach. The patient drinks a liquid that contains barium (a silver-white metallic compound). The liquid coats the esophagus and stomach, and x-rays are taken. This procedure is also called an upper GI series.
- Esophagoscopy: A procedure to look inside the esophagus to check for abnormalareas. An esophagoscope is inserted through the mouth or nose and down the throat into the esophagus. An esophagoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer. A biopsy is usually done during an esophagoscopy. Sometimes a biopsy shows changes in the esophagus that are not cancer but may lead to cancer.
- Bronchoscopy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
- Thoracoscopy: A surgical procedure to look at the organs inside the chest to check for abnormal areas. An incision (cut) is made between two ribs and a thoracoscope is inserted into the chest. A thoracoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. Sometimes this procedure is used to remove part of the esophagus or lung.
Prognosis
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of esophageal cancer in children may include the following:
- Radiation therapy given through a plastic or metal tube placed through the mouth into the esophagus.
- Chemotherapy.
- Surgery to remove all or part of the tumor.
Treatment of recurrent esophageal cancer in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
See the PDQ summary on adult Esophageal Cancer for more information.
Thymoma
Thymoma is a tumor of the cells that cover the outside surface of the thymus. The thymus is a small organ in the upper chest under the breastbone. It is part of the lymph systemand makes white blood cells, called lymphocytes, that help fight infection. Thymoma usually forms between the lungs in the front part of the chest and is often found during a chest x-ray that is done for another reason.
The thymoma tumor cells look a lot like the normal cells of the thymus, grow slowly, and rarely spread beyond the thymus.
Other types of tumors, such as lymphoma or germ cell tumors, may form in the thymus but they are not considered to be thymoma.
Signs and Symptoms and Diagnostic and Staging Tests
Thymoma may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:
- Coughing.
- Pain or a tight feeling in the chest.
- Trouble breathing.
- Trouble swallowing.
- Hoarseness.
- Fever.
- Weight loss.
- Superior vena cava syndrome.
Other conditions that are not thymoma may cause these same signs and symptoms.
People who develop thymoma often have one of the following immune system diseases or hormone disorders:
- Myasthenia gravis.
- Pure red cell aplasia.
- Hypogammaglobulinemia.
- Nephrotic syndrome.
- Scleroderma.
- Dermatomyositis.
- Lupus.
- Rheumatoid arthritis.
- Thyroiditis.
- Hyperthyroidism.
- Addison disease.
- Panhypopituitarism.
- Physical exam and health history.
- X-ray of the chest.
- CT scan.
- PET scan.
- MRI.
- Biopsy.
See the General Information section for a description of these tests and procedures.
Prognosis
The prognosis (chance of recovery) is better when the tumor has not spread to other parts of the body. Childhood thymoma is usually diagnosed before the tumor has spread.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of thymoma in children may include the following:
- Surgery to remove as much of the tumor as possible.
- Radiation therapy, for tumors that cannot be removed by surgery or if tumor remains after surgery.
- Chemotherapy, for tumors that did not respond to other treatments.
- Hormone therapy (octreotide), for tumors that did not respond to other treatments.
- Targeted therapy (sunitinib), for tumors that did not respond to other treatments.
Treatment of recurrent thymoma in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
See the PDQ summary on adult Thymoma and Thymic Carcinoma Treatment for more information.
Thymic Carcinoma
Thymic carcinoma is a cancer of the cells that cover the outside surface of the thymus. The thymus is a small organ in the upper chest under the breastbone. It is part of the lymph system and makes white blood cells, called lymphocytes, that help fight infection. Thymic carcinoma usually forms between the lungs in the front part of the chest and is often found during a chest x-ray that is done for another reason.
The tumor cells in thymic carcinoma do not look like the normal cells of the thymus, grow more quickly, and are more likely to spread to other parts of the body.
Other types of tumors, such as lymphoma or germ cell tumors, may form in the thymus but they are not considered to be thymic carcinoma. (See the Thymoma section above for more information).
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Thymic carcinoma can rarely be completely removed by surgery and is likely to recur (come back) after treatment.
Treatment of thymic carcinoma in children may include the following:
- Surgery to remove as much of the tumor as possible.
- Radiation therapy, for tumors that cannot be removed by surgery or if tumor remains after surgery.
- Chemotherapy, for tumors that did not respond to radiation therapy.
- Targeted therapy (sunitinib), for tumors that did not respond to other treatments.
Treatment of recurrent thymic carcinoma in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
See the PDQ summary on adult Thymoma and Thymic Carcinoma Treatment for more information.
Heart Tumors
Most tumors that form in the heart are benign (not cancer). Benign heart tumors that may appear in children include the following:
- Rhabdomyoma: A tumor that forms in muscle made up of long fibers.
- Myxoma: A tumor that may be part of an inherited syndrome called Carney complex. (See the Multiple Endocrine Neoplasia Syndromes section for more information.)
- Teratomas: A type of germ cell tumor. In the heart, these tumors form most often in the pericardium (the sac that covers the heart). Some teratomas are malignant(cancer).
- Fibroma: A tumor that forms in fiber-like tissue that holds bones, muscles, and other organs in place.
- Histiocytoid cardiomyopathy tumor: A tumor that forms in the heart cells that control heart rhythm.
- Hemangiomas: A tumor that forms in the cells that line blood vessels.
- Neurofibroma: A tumor that forms in the cells and tissues that cover nerves.
Before birth and in newborns, the most common benign heart tumors are teratomas. An inherited condition called tuberous sclerosis can cause heart tumors to form in an unborn baby (fetus) or newborn.
Malignant tumors that begin in the heart are even more rare than benign heart tumors in children. Malignant heart tumors include:
- Malignant teratoma.
- Lymphoma.
- Rhabdomyosarcoma: A cancer that forms in muscle made up of long fibers.
- Angiosarcoma: A cancer that forms in cells that line blood vessels or lymph vessels.
- Undifferentiated pleomorphic sarcoma: A cancer that usually forms in the soft tissue, but it may also form in bone.
- Leiomyosarcoma: A cancer that forms in smooth muscle cells.
- Chondrosarcoma: A cancer that usually forms in bone cartilage but very rarely can begin in the heart.
- Synovial sarcoma: A cancer that usually forms around joints but may very rarely form in the heart or sac around the heart.
- Infantile fibrosarcoma: A cancer that forms in fiber-like tissue that holds bones, muscles, and other organs in place.
Signs and Symptoms
Heart tumors may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:
- Change in the heart's normal rhythm.
- Trouble breathing, especially when the child is lying down.
- Pain or tightness in the middle of the chest that feels better when the child is sitting up.
- Coughing.
- Fainting.
- Feeling dizzy, tired, or weak.
- Fast heart rate.
- Swelling in the legs, ankles, or abdomen.
- Feeling anxious.
- Signs of a stroke.
- Sudden numbness or weakness of the face, arm, or leg (especially on one side of the body).
- Sudden confusion or trouble speaking or understanding.
- Sudden trouble seeing with one or both eyes.
- Sudden trouble walking or feeling dizzy.
- Sudden loss of balance or coordination.
- Sudden severe headache for no known reason.
Sometimes heart tumors do not cause any signs or symptoms.
Other conditions that are not heart tumors may cause these same signs and symptoms.
Diagnostic and Staging Tests
- Physical exam and health history.
- X-ray of the chest.
- CT scan.
- MRI of the heart.
See the General Information section for a description of these tests and procedures.
Other tests used to diagnose or stage heart tumors include the following:
- Echocardiogram: A procedure in which high-energy sound waves (ultrasound) are bounced off the heart and nearby tissues or organs and make echoes. A moving picture is made of the heart and heart valves as blood is pumped through the heart.
- Electrocardiogram (EKG): A recording of the heart's electrical activity to check its rate and rhythm. A number of small pads (electrodes) are placed on the patient’s chest, arms, and legs, and are connected by wires to the EKG machine. Heart activity is then recorded as a line graph on paper. Electrical activity that is faster or slower than normal may be a sign of heart disease or damage.
- Cardiac catheterization: A procedure to look inside the blood vessels and heart for abnormal areas or cancer. A long, thin, catheter is inserted into an artery or vein in the groin, neck, or arm and threaded through the blood vessels to the heart. A sample of tissue may be removed using a special tool. A pathologist views the tissue under a microscope to look for cancer cells.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of heart tumors in children may include the following:
- Watchful waiting, for rhabdomyoma, which sometimes shrinks and goes away on its own.
- Targeted therapy (everolimus) for patients who have rhabdomyoma and tuberous sclerosis.
- Chemotherapy followed by surgery (which may include removing some or all of the tumor or a heart transplant), for sarcomas.
- Surgery alone, for other tumor types.
- Radiation therapy for tumors that cannot be removed by surgery.
Treatment of recurrent heart tumors in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
Mesothelioma
The tumors often spread over the surface of organs without spreading into the organ. They may spread to nearby lymph nodes or in other parts of the body. Malignant mesothelioma may also form in the testicles, but this is rare.
Risk Factors and Signs and Symptoms
Mesothelioma is sometimes a late effect of treatment for an earlier cancer, especially after treatment with radiation therapy. In adults, mesothelioma is linked to being exposed to asbestos, which was once used as building insulation. There is no information about the risk of mesothelioma in children exposed to asbestos.
Mesothelioma may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:
- Trouble breathing.
- Cough for no known reason.
- Pain under the rib cage or pain in the chest and abdomen.
- Weight loss for no known reason.
- Feeling very tired.
Other conditions that are not mesothelioma may cause these same signs and symptoms.
Diagnostic and Staging Tests
- Physical exam and health history.
- X-ray of the chest.
- CT scan.
- PET scan.
- MRI.
- Fine-needle aspiration (FNA) biopsy.
See the General Information section for a description of these tests and procedures.
Other tests used to diagnose mesothelioma include the following:
- Pulmonary function test (PFT): A test to see how well the lungs are working. It measures how much air the lungs can hold and how quickly air moves into and out of the lungs. It also measures how much oxygen is used and how much carbon dioxide is given off during breathing. This is also called a lung function test.
- Bronchoscopy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
- Thoracoscopy: A surgical procedure to look at the organs inside the chest to check for abnormal areas. An incision (cut) is made between two ribs and a thoracoscope is inserted into the chest. A thoracoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. In some cases, this procedure is used to remove part of the esophagus or lung.
- Laparoscopy: A surgical procedure to look at the organs inside the abdomen to check for abnormal areas. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (thin, lighted tube) is inserted into one of the incisions. Other instruments may be inserted through the same or other incisions to perform procedures such as removing organs or taking tissue samples to be checked under a microscope for signs of cancer.
- Cytologic exam: An exam of cells under a microscope (by a pathologist) to check for anything abnormal. For mesothelioma, fluid is taken from around the lungs or from the abdomen. A pathologist checks the cells in the fluid.
Prognosis
The prognosis (chance of recovery) is better when the tumor has not spread to other parts of the body.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of mesothelioma in children may include the following:
- Surgery to remove the part of the chest lining with cancer and some of the healthy tissue around it.
- Chemotherapy.
- Radiation therapy, as palliative therapy, to relieve pain and improve quality of life.
Treatment of recurrent mesothelioma in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
See the PDQ summary on adult Malignant Mesothelioma Treatment for more information.
Unusual Cancers of the Abdomen
Adrenocortical Carcinoma
Adrenocortical carcinoma is a disease in which malignant (cancer) cells form in the outer layer of the adrenal gland. There are two adrenal glands. The adrenal glands are small and shaped like a triangle. One adrenal gland sits on top of each kidney. Each adrenal gland has two parts. The center of the adrenal gland is the adrenal medulla. The outer layer of the adrenal gland is the adrenal cortex. Adrenocortical carcinoma is also called cancer of the adrenal cortex.
Childhood adrenocortical carcinoma occurs most commonly in patients younger than 6 years or in the teen years, and more often in females.
The adrenal cortex makes important hormones that do the following:
- Balance the water and salt in the body.
- Help keep blood pressure normal.
- Help control the body's use of protein, fat, and carbohydrates.
- Cause the body to have male or female characteristics.
Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests
The risk of adrenocortical carcinoma is increased by having a certain mutation (change) in a gene or any of the following syndromes:
Adrenocortical carcinoma may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:
- A lump in the abdomen.
- Pain in the abdomen or back.
- Feeling of fullness in the abdomen.
Also, a tumor of the adrenal cortex may be functioning (makes more hormones than normal) or nonfunctioning (does not make extra hormones). Most tumors of the adrenal cortex in children are functioning tumors. The extra hormones made by functioning tumors may cause certain signs or symptoms of disease and these depend on the type of hormone made by the tumor. For example, extra androgen hormone may cause both male and female children to develop masculine traits, such as body hair or a deep voice, grow faster, and have acne. Extra estrogen hormone may cause the growth of breast tissue in male children. Extra cortisol hormone may cause Cushing syndrome (hypercortisolism).
(See the PDQ summary on adult Adrenocortical Carcinoma Treatment for more information on the signs and symptoms of adrenocortical carcinoma.)
The tests and procedures used to diagnose and stage adrenocortical carcinoma depend on the patient's symptoms. These tests and procedures may include the following:
- Physical exam and health history.
- Blood chemistry studies.
- X-ray of the chest, abdomen, or bones.
- CT scan.
- MRI.
- PET scan.
- Ultrasound.
- Biopsy (the mass is removed during surgery and then the sample is checked for signs of cancer).
See the General Information section for a description of these tests and procedures.
Other tests used to diagnose adrenocortical carcinoma include the following:
- Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of cortisol or 17-ketosteroids. A higher than normal amount of these substances in the urine may be a sign of disease in the adrenal cortex.
- Low-dose dexamethasone suppression test: A test in which one or more small doses of dexamethasone are given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days. This test is done to check if the adrenal gland is making too much cortisol.
- High-dose dexamethasone suppression test: A test in which one or more high doses of dexamethasone are given. The level of cortisol is checked from a sample of blood or from urine that is collected for three days. This test is done to check if the adrenal gland is making too much cortisol or if the pituitary gland is telling the adrenal glands to make too much cortisol.
- Blood hormone studies: A procedure in which a blood sample is checked to measure the amounts of certain hormones released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. The blood may be checked for testosterone or estrogen. A higher than normal amount of these hormones may be a sign of adrenocortical carcinoma.
- Adrenal angiography: A procedure to look at the arteries and the flow of blood near the adrenal gland. A contrast dye is injected into the adrenal arteries. As the dye moves through the blood vessel, a series of x-rays are taken to see if any arteries are blocked.
- Adrenal venography: A procedure to look at the adrenal veins and the flow of blood near the adrenal glands. A contrast dye is injected into an adrenal vein. As the contrast dye moves through the vein, a series of x-rays are taken to see if any veins are blocked. A catheter (very thin tube) may be inserted into the vein to take a blood sample, which is checked for abnormal hormone levels.
Prognosis
The prognosis (chance of recovery) is good for patients who have small tumors that have been completely removed by surgery. For other patients, the prognosis depends on the following:
- Size of the tumor.
- How quickly the cancer is growing.
- Whether there are changes in certain genes.
- Whether the tumor has spread to other parts of the body, including the lymph nodes.
- Child's age.
- Whether the covering around the tumor broke open during surgery to remove the tumor.
- Whether the tumor was completely removed during surgery.
- Whether the child has developed masculine traits.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of adrenocortical carcinoma in children may include the following:
- Surgery to remove the adrenal gland and, if needed, cancer that has spread to other parts of the body. Sometimes chemotherapy is also given.
Treatment of recurrent adrenocortical carcinoma in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
See the PDQ summary on adult Adrenocortical Carcinoma Treatment for more information.
Stomach (Gastric) Cancer
Stomach cancer is a disease in which malignant (cancer) cells form in the lining of the stomach. The stomach is a J-shaped organ in the upper abdomen. It is part of the digestive system, which processes nutrients (vitamins, minerals, carbohydrates, fats, proteins, and water) in foods that are eaten and helps pass waste material out of the body. Food moves from the throat to the stomach through a hollow, muscular tube called the esophagus. After leaving the stomach, partly-digested food passes into the small intestine and then into the large intestine.
Risk Factors and Signs and Symptoms
The risk of stomach cancer is increased by the following:
- Having an infection with Helicobacter pylori (H. pylori) bacterium, which is found in the stomach.
- Having an inherited condition called familial diffuse gastric cancer.
Many patients do not have signs and symptoms until the cancer spreads. Stomach cancer may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:
Other conditions that are not stomach cancer may cause these same signs and symptoms.
Diagnostic and Staging Tests
- Physical exam and health history.
- X-ray of the abdomen.
- Blood chemistry studies.
- CT scan.
- Biopsy.
See the General Information section for a description of these tests and procedures.
Other tests used to diagnose and stage stomach cancer include the following:
- Upper endoscopy: A procedure to look inside the esophagus, stomach, and duodenum(first part of the small intestine) to check for abnormal areas. An endoscope is passed through the mouth and down the throat into the esophagus. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease.
- Barium swallow: A series of x-rays of the esophagus and stomach. The patient drinks a liquid that contains barium (a silver-white metallic compound). The liquid coats the esophagus and stomach, and x-rays are taken. This procedure is also called an upper GI series.
- Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
- The number of red blood cells, white blood cells, and platelets.
- The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
- The portion of the blood sample made up of red blood cells.
Prognosis
Prognosis (chance of recovery) depends on whether the cancer has spread at the time of diagnosis and how well the cancer responds to treatment.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of stomach cancer in children may include the following:
- Surgery to remove the cancer and some healthy tissue around it.
- Surgery to remove as much of the cancer as possible, followed by radiation therapyand chemotherapy.
Treatment of recurrent stomach cancer in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
See the Gastrointestinal Stromal Tumors (GIST) section of this summary and the Neuroendocrine Tumors (Carcinoids) section of this summary for information about gastrointestinal carcinoids and neuroendocrine tumors.
Pancreatic Cancer
Pancreatic cancer is a disease in which malignant (cancer) cells form in the tissues of the pancreas. The pancreas is a pear-shaped gland about 6 inches long. The wide end of the pancreas is called the head, the middle section is called the body, and the narrow end is called the tail. Many different kinds of tumors can form in the pancreas. Some tumors are benign (not cancer).
The pancreas has two main jobs in the body:
- To make juices that help digest (break down) food. These juices are secreted into the small intestine.
- To make hormones that help control the sugar and salt levels in the blood. These hormones are secreted into the bloodstream.
There are four types of pancreatic cancer in children:
- Solid pseudopapillary tumor of the pancreas. This is the most common type of pancreatic tumor. It most commonly affects females that are older adolescents and young adults. These slow-growing tumors have both cyst-like and solid parts. Solid pseudopapillary tumor of the pancreas is unlikely to spread to other parts of the body and the prognosis is very good. Occasionally, the tumor may spread to the liver, lung, or lymph nodes.
- Pancreatoblastoma. It usually occurs in children aged 10 years or younger. Children with Beckwith-Wiedemann syndrome and familial adenomatous polyposis (FAP) syndrome have an increased risk of developing pancreatoblastoma. These slow-growing tumors often make the tumor marker alpha-fetoprotein. These tumors may also make adrenocorticotropic hormone (ACTH) and antidiuretic hormone (ADH). Pancreatoblastoma may spread to the liver, lungs, and lymph nodes. The prognosis for children with pancreatoblastoma is good.
- Islet cell tumors. These tumors are not common in children and can be benign or malignant. Islet cell tumors may occur in children with multiple endocrine neoplasia type 1 (MEN1) syndrome. The most common types of islet cell tumors are insulinomasand gastrinomas. Other types of islet cell tumors are ACTHoma and VIPoma. These tumors may make hormones, such as insulin, gastrin, ACTH, or ADH. When too much of a hormone is made, signs and symptoms of disease occur.
- Pancreatic carcinoma. Pancreatic carcinoma is very rare in children. The two types of pancreatic carcinoma are acinar cell carcinoma and ductal adenocarcinoma.
Signs and Symptoms
General signs and symptoms of pancreatic cancer may include the following:
In children, some pancreatic tumors do not secrete hormones and there are no signs and symptoms of disease. This makes it hard to diagnose pancreatic cancer early.
Pancreatic tumors that do secrete hormones may cause signs and symptoms. The signs and symptoms depend on the type of hormone being made.
If the tumor secretes insulin, signs and symptoms that may occur include the following:
- Low blood sugar. This can cause blurred vision, headache, and feeling lightheaded, tired, weak, shaky, nervous, irritable, sweaty, confused, or hungry.
- Changes in behavior.
- Seizures.
- Coma.
If the tumor secretes gastrin, signs and symptoms that may occur include the following:
- Stomach ulcers that keep coming back.
- Pain in the abdomen, which may spread to the back. The pain may come and go and it may go away after taking an antacid.
- The flow of stomach contents back into the esophagus (gastroesophageal reflux).
- Diarrhea.
Signs and symptoms caused by tumors that make other types of hormones, such as ACTH or ADH, may include the following:
- Watery diarrhea.
- Dehydration (feeling thirsty, making less urine, dry skin and mouth, headaches, dizziness, or feeling tired).
- Low sodium (salt) level in the blood (confusion, sleepiness, muscle weakness, and seizures).
- Weight loss or gain for no known reason.
- Round face and thin arms and legs.
- Feeling very tired and weak.
- High blood pressure.
- Purple or pink stretch marks on the skin.
Check with your child’s doctor if you see any of these problems in your child. Other conditions that are not pancreatic cancer may cause these same signs and symptoms.
Diagnostic and Staging Tests
Tests to diagnose and stage pancreatic cancer may include the following:
- Physical exam and health history.
- X-ray of the chest.
- CT scan.
- MRI.
- PET scan.
- Biopsy.
- Core-needle biopsy: The removal of tissue using a wide needle.
- Laparoscopy: A surgical procedure to look at the organs inside the abdomen to check for signs of disease. Small incisions (cuts) are made in the wall of the abdomen and a laparoscope (a thin, lighted tube) is inserted into one of the incisions. Other instruments may be inserted through the same or other incisions to perform procedures such as removing organs or taking tissue samples to be checked under a microscope for signs of disease.
- Laparotomy: A surgical procedure in which an incision (cut) is made in the wall of the abdomen to check the inside of the abdomen for signs of disease. The size of the incision depends on the reason the laparotomy is being done. Sometimes organs are removed or tissue samples are taken and checked under a microscope for signs of disease.
See the General Information section for a description of these tests and procedures.
Other tests used to diagnose pancreatic cancer include the following:
- Endoscopic ultrasound (EUS): A procedure in which an endoscope is inserted into the body, usually through the mouth or rectum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. A probe at the end of the endoscope is used to bounce high-energy sound waves (ultrasound) off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. This procedure is also called endosonography.
- Somatostatin receptor scintigraphy: A type of radionuclide scan used to find pancreatic tumors. A very small amount of radioactive octreotide (a hormone that attaches to carcinoid tumors) is injected into a vein and travels through the bloodstream. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is used to diagnose islet cell tumors.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of solid pseudopapillary tumor of the pancreas in children may include the following:
- Surgery to remove the tumor.
- Chemotherapy for tumors that cannot be removed by surgery or have spread to other parts of the body.
Treatment of pancreatoblastoma in children may include the following:
- Surgery to remove the tumor. A Whipple procedure may be done for tumors in the head of the pancreas.
- Chemotherapy may be given to shrink the tumor before surgery. More chemotherapy may be given after surgery for large tumors, tumors that could not initially be removed by surgery, and tumors that have spread to other parts of the body.
- Chemotherapy may be given if the tumor does not respond to treatment or comes back.
Treatment of islet cell tumors in children may include drugs to treat symptoms caused by hormones and the following:
- Surgery to remove the tumor.
- Chemotherapy and targeted therapy (mTOR inhibitor therapy) for tumors that cannot be removed by surgery or that have spread to other parts of the body.
See the PDQ summary on adult Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment for more information on pancreatic tumors.
There are few reported cases of pancreatic carcinoma in children. (See the PDQ summary on adult Pancreatic Cancer Treatment for possible treatment options.)
Treatment of recurrent pancreatic carcinoma in children may include the following:
- A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
See the PDQ summaries on adult Pancreatic Cancer Treatment and adult Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment for more information on pancreatic tumors.
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