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Cardiopulmonary Syndromes (PDQ®)

Patient Version
Last Modified: 08/19/2011

Superior Vena Cava Syndrome in Children

Key Points for This Section


Superior vena cava syndrome in a child is a serious medical emergency because the child's windpipe can become blocked.

Superior vena cava syndrome (SVCS) in children can be life-threatening. This is because the trachea (windpipe) can quickly become blocked. In adults, the windpipe is fairly stiff, but in children, it is softer and can more easily be squeezed shut. Also, a child's windpipe is narrower, so any amount of swelling can cause breathing problems. Squeezing of the trachea is called superior mediastinal syndrome (SMS). Because SVCS and SMS usually happen together in children, the two syndromes are considered to be the same.

The most common symptoms of SVCS in children are a lot like those in adults.

Common symptoms include the following:

  • Coughing.
  • Hoarseness.
  • Trouble breathing.
  • Chest pain.

There are other less common but more serious symptoms:

  • Fainting.
  • Anxiety.
  • Confusion.
  • Headache.
  • Vision problems.
  • A feeling of fullness in the ears.

The causes, diagnosis, and treatment of SVCS in children are not the same as in adults.

The most common cause of SVCS in children is non-Hodgkin lymphoma.

SVCS in children is rare. The most common cause is non-Hodgkin lymphoma. As in adults, SVCS may also be caused by a blood clot that forms during use of an intravenous catheter (flexible tube used to put fluids into or take blood out of a vein) in the superior vena cava.

SVCS in children may be diagnosed and treated before a diagnosis of cancer is made.

A physical exam, chest x-ray, and medical history are usually all that are needed to diagnose superior vena cava syndrome in children. Even if doctors think cancer is causing SVCS, a biopsy may not be done. This is because the lungs and heart of a child with SVCS may not be able to handle the anesthesia needed. Other imaging tests may be done to help find out if anesthesia can be safely used. In most cases, treatment will begin before a diagnosis of cancer is made.

The following treatments may be used for SVCS in children:

  • Radiation therapy

    Radiation therapy is usually used to treat a tumor that is blocking the vein. After radiation therapy, there may be more trouble breathing because swelling narrows the windpipe. A drug to reduce swelling may be given.

  • Drugs

    Anticancer drugs, steroids, or other drugs may be used. If the tumor does not respond, it may be benign (not cancer).

  • Surgery

    This may include surgery to bypass (go around) the blocked part of the vein or to place a stent (thin tube) to open the vein.



Glossary Terms

anesthesia (A-nes-THEE-zhuh)
A loss of feeling or awareness caused by drugs or other substances. Anesthesia keeps patients from feeling pain during surgery or other procedures. Local anesthesia is a loss of feeling in one small area of the body. Regional anesthesia is a loss of feeling in a part of the body, such as an arm or leg. General anesthesia is a loss of feeling and a complete loss of awareness that feels like a very deep sleep.
anxiety (ang-ZY-eh-tee)
Feelings of fear, dread, and uneasiness that may occur as a reaction to stress. A person with anxiety may sweat, feel restless and tense, and have a rapid heart beat. Extreme anxiety that happens often over time may be a sign of an anxiety disorder.
benign (beh-NINE)
Not cancerous. Benign tumors may grow larger but do not spread to other parts of the body. Also called nonmalignant.
biopsy (BY-op-see)
The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.
blood (blud)
A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.
blood clot (blud klot)
A mass of blood that forms when blood platelets, proteins, and cells stick together. When a blood clot is attached to the wall of a blood vessel, it is called a thrombus. When it moves through the bloodstream and blocks the flow of blood in another part of the body, it is called an embolus.
bypass (BY-pas)
A surgical procedure in which the doctor creates a new pathway for the flow of body fluids.
cancer (KAN-ser)
A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord. Also called malignancy.
catheter (KA-theh-ter)
A flexible tube used to deliver fluids into or withdraw fluids from the body.
chest x-ray (chest EX-ray)
An x-ray of the structures inside the chest. An x-ray is a type of high-energy radiation that can go through the body and onto film, making pictures of areas inside the chest, which can be used to diagnose disease.
confusion (kun-FYOO-zhun)
A mental state in which one is not thinking clearly.
diagnosis (DY-ug-NOH-sis)
The process of identifying a disease, such as cancer, from its signs and symptoms.
drug (drug)
Any substance, other than food, that is used to prevent, diagnose, treat or relieve symptoms of a disease or abnormal condition. Also refers to a substance that alters mood or body function, or that can be habit-forming or addictive, especially a narcotic.
fluid (FLOO-id)
A substance that flows smoothly and takes the shape of its container. Liquids and gases are fluids.
imaging (IH-muh-jing)
In medicine, a process that makes pictures of areas inside the body. Imaging uses methods such as x-rays (high-energy radiation), ultrasound (high-energy sound waves), and radio waves.
intravenous (IN-truh-VEE-nus)
Into or within a vein. Intravenous usually refers to a way of giving a drug or other substance through a needle or tube inserted into a vein. Also called IV.
lung (lung)
One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.
mediastinum (MEE-dee-uh-STY-num)
The area between the lungs. The organs in this area include the heart and its large blood vessels, the trachea, the esophagus, the thymus, and lymph nodes but not the lungs.
medical history (MEH-dih-kul HIH-stuh-ree)
A record of information about a person’s health. A personal medical history may include information about allergies, illnesses, surgeries, immunizations, and results of physical exams and tests. It may also include information about medicines taken and health habits, such as diet and exercise. A family medical history includes health information about a person’s close family members (parents, grandparents, children, brothers, and sisters). This includes their current and past illnesses. A family medical history may show a pattern of certain diseases in a family.
non-Hodgkin lymphoma (non-HOJ-kin lim-FOH-muh)
Any of a large group of cancers of lymphocytes (white blood cells). Non-Hodgkin lymphomas can occur at any age and are often marked by lymph nodes that are larger than normal, fever, and weight loss. There are many different types of non-Hodgkin lymphoma. These types can be divided into aggressive (fast-growing) and indolent (slow-growing) types, and they can be formed from either B-cells or T-cells. B-cell non-Hodgkin lymphomas include Burkitt lymphoma, chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), diffuse large B-cell lymphoma, follicular lymphoma, immunoblastic large cell lymphoma, precursor B-lymphoblastic lymphoma, and mantle cell lymphoma. T-cell non-Hodgkin lymphomas include mycosis fungoides, anaplastic large cell lymphoma, and precursor T-lymphoblastic lymphoma. Lymphomas that occur after bone marrow or stem cell transplantation are usually B-cell non-Hodgkin lymphomas. Prognosis and treatment depend on the stage and type of disease. Also called NHL.
physical examination (FIH-zih-kul eg-ZA-mih-NAY-shun)
An exam of the body to check for general signs of disease.
radiation therapy (RAY-dee-AY-shun THAYR-uh-pee)
The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called irradiation and radiotherapy.
response (reh-SPONTS)
In medicine, an improvement related to treatment.
stent (stent)
A device placed in a body structure (such as a blood vessel or the gastrointestinal tract) to keep the structure open.
steroid drug (STAYR-oyd ...)
A type of drug used to relieve swelling and inflammation. Some steroid drugs may also have antitumor effects.
superior vena cava (soo-PEER-ee-er VEE-nuh KAY-vuh)
The large vein that carries blood from the head, neck, arms, and chest to the heart.
superior vena cava syndrome (soo-PEER-ee-er VEE-nuh KAY-vuh SIN-drome)
A condition in which a tumor presses against the superior vena cava (the large vein that carries blood from the head, neck, arms, and chest to the heart). This pressure blocks blood flow to the heart and may cause coughing, difficulty in breathing, and swelling of the face, neck, and upper arms.
surgery (SER-juh-ree)
A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.
symptom (SIMP-tum)
An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.
syndrome (SIN-drome)
A set of symptoms or conditions that occur together and suggest the presence of a certain disease or an increased chance of developing the disease.
trachea (TRAY-kee-uh)
The airway that leads from the larynx (voice box) to the bronchi (large airways that lead to the lungs). Also called windpipe.
tumor (TOO-mer)
An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.
vein (vayn)
A blood vessel that carries blood to the heart from tissues and organs in the body.
windpipe (WIND-pipe)
The airway that leads from the larynx (voice box) to the bronchi (large airways that lead to the lungs). Also called trachea.