What's to know about Pancoast syndrome?
A Pancoast tumor is a non-small cell tumor that has spread and affected nearby tissues, specifically the vertebrae and the ribs. Another name for it is a superior sulcus tumor.
As the tumor spreads, it triggers the neurological symptoms that make it different than other types of cancer.
Pancoast tumors represent fewer than 5% of all lung tumors. The tumor and the syndrome take their names from the American radiologist who discovered them in 1932 — Dr. Henry Pancoast.
What are Pancoast tumors?
Pancoast tumors develop at the top of either lung.
Image credit: Jmarchn, 2010
When a person develops a tumor in certain parts of the body — usually the top of the lung — it can spread to nearby tissues and cause a range of symptoms.
Doctors can distinguish Pancoast tumors from other tumors because they grow at the top of either lung and lead to neurological symptoms.
Pancoast tumors develop from non-small cell cancers. They usually start as a squamous cell cancer, but can also sometimes begin as an adenocarcinoma or large cell cancer.
These tumors affect other structures, including the:
- lymph vessels
- nearby ribs
- region between the lungs
- small bones of the spine
- smoking tobacco or exposure to secondhand tobacco smoke
- age, as most appear when people are between the ages of 50 and 60 years
- sex, as they tend to affect males more often than females
Symptoms have also arisen with cancers that have spread from the larynx, thyroid, bladder, and cervix.
In rare cases, there has been evidence that symptoms stem from bacterial and fungal infections, such as tuberculosis and aspergillosis.
The symptoms of a Pancoast tumor differ from those of cancer in other parts of the lung. Due to their position at the top of the lung, they rarely cause the coughing and shortness of breath that usually occur with lung cancer.
Instead, the tumor puts pressure on the nerves. This pressure can lead to pain in the upper chest, neck, face, or arms, and it can also lead to the following symptoms:
- severe and persistent pain in the shoulder and shoulder blade
- radiating pain in the arm or chest wall
- pain, tingling, or weakness in the hand and fingers
The pain may be severe and constant, and the hand or arm may waste away or shrink in size. The extent of the tumor's effects will depend on where it grows.
As cancer progresses, it may start to metastasize (spread) throughout the body. The person will begin to experience fatigue, a loss of appetite, and other symptoms that are common with cancer.
Horner's syndrome can also occur with Pancoast tumors. Symptoms result from damage or disruption to certain nerves.
Horner's syndrome typically affects only one side of the face.
A person may notice the following:
- the pupil on the affected side shrinking in size, called pinpoint pupil
- the upper eyelid starting to droop
- flushing and an inability to sweat
Diagnosis of Pancoast syndrome and Pancoast tumors can take time because the symptoms are not typical of lung cancer.
Many people will see a neurologist or orthopedic surgeon before consulting an oncologist.
A doctor will diagnose a Pancoast tumor using:
X-ray: This scan may reveal abnormal tissue at the top of the lung. It can also show whether the tumor has invaded the ribs or vertebrae. However, in the early stages, shadowing may make Pancoast tumors difficult to see on an X-ray.
CT or MRI scan: These scans can indicate whether the tumor has entered the chest wall, spine, blood vessels, nerves, windpipe, esophagus (food pipe), or region between the lungs.
Bronchoscopy: For this procedure, a doctor will use a lighted tube with a camera on it to view the airways.
Tissue biopsy: If a doctor suspects cancer, they will perform a biopsy. This procedure involves removing a sample of cells for examination. Usually, they will do this by passing a needle through the skin.
If the biopsy results confirm that cancer is present, the doctor may carry out further tests to see whether cancer has spread to other areas of the body, such as the brain or bones.
The doctor will also need to know the stage of the cancer. This information will help them decide on suitable treatment options and estimate the individual's outlook.
Doctors use the TNM system to stage Pancoast tumors. This system focuses on the following factors:
Tumor (T): How big is the tumor?
Nodes (N): Has it affected lymph nodes?
Metastasis (M): Has it spread to more distant parts of the body?
The doctor assigns a number to each factor. The higher the number, the more advanced the cancer is, and the harder it is to treat.
Pancoast tumors are tumors that have spread and affected other tissues. For this reason, their stage will always be T3 or T4 in the TNM staging system.
T3 tumors affect only the chest wall or the chain of nerves that runs from the neck down the spine.
T4 tumors have reached either the spine, the nerves running from the neck to the upper limbs, or the blood vessels.
When to see a doctor
Anyone who develops symptoms of Pancoast syndrome or any type of lung cancer should see a doctor.
Pancoast tumors are uncommon. To be sure of an accurate diagnosis, a person may wish to consult a doctor with specialist knowledge of lung cancer, including Pancoast syndrome.
Surgery is one possible treatment for Pancoast syndrome.
The type of treatment will depend on the overall health of the individual, as well as the size of the tumor and the areas of the body that it affects.
Treatment can aim to reduce symptoms or to remove the cancer, and it may involve one or more of the following:
Options for managing the symptoms include pain relief medication and steroid treatment to reduce nerve pressure.
Fewer than half of those with a Pancoast tumor can have surgery. In many cases, surgery is not possible due to the spread of the tumor.
The type of surgery will depend on which tissues the tumor has affected. The surgeon may need to remove the entire lung, affected nearby tissues, or the top ribs.
In some cases, they may need to remove a major artery and replace it with an artificial tube to maintain blood supply.
Surgery for Pancoast tumors can be risky. It carries an overall mortality rate of 5% and a 7–38% chance of complications.
Possible complications include:
- a risk of infection
- an air embolism
- vascular complications
- neurological damage, leading, for example, to weakness in the hand
Experts base these estimates on past statistics, however. Individual factors — such as overall health and age — always play a role in a person's outcome following surgery or treatment.
Chemotherapy and radiation therapy
A doctor may recommend chemotherapy, radiation therapy, or a combination of the two, sometimes called "chemoradiotherapy."
Chemotherapy involves the use of special drugs, while radiation therapy works by targeting cancer cells with an external beam or a radioactive substance that the surgeon implants in the body.
Both techniques can kill cancer cells, but they can have adverse side effects.
Studies have suggested that a combination of chemotherapy and radiation therapy may be more effective than chemotherapy alone, although the adverse effects may be more severe.
New approaches, including drugs known as biologic agents, affect factors that lead more specifically to cancer. In the future, they may improve the outcome for people with Pancoast syndrome.
The most important preventive measure to reduce the risk of any lung cancer is to quit smoking, if applicable, and to try to avoid inhaling secondhand smoke.
Screening can help doctors detect changes when cancer is in the early stages, allowing for early treatment, which may lead to a better outcome.
People who might benefit from screening are those who:
- have a family history of lung cancer
- have a history of exposure to asbestos
- are over 55 years of age and have a history of smoking
The outlook for a Pancoast tumor has improved in recent decades with medical advances.
In the past, a Pancoast tumor was always fatal, but medical advances in the last few decades have improved the outlook for people with this cancer.
After treatment with chemotherapy, radiation, and surgery, the overall 2 year survival rate is 55–70%.
If surgery allows the complete removal of cancerous cells, the 5 year survival rate is 54–77%. The outlook is better for people with T3 stage tumors than for those with T4 stage tumors.
The outlook is less positive for people who:
- receive a diagnosis at the T4 stage
- also have Horner's syndrome
- have cancerous cells that doctors cannot remove
Pancoast tumors are treatable, but there is also a risk that they will come back. If they do, they tend to affect the brain.
Anyone who notices neurological or other symptoms that could result from a Pancoast tumor should see a doctor as soon as possible.
It is especially important for them to do this if they have a high risk of lung cancer, due, for example, to a history of smoking tobacco.
Anyone who is considering quitting smoking should speak to their doctor, who will be able to help. Products for quitting smoking are available in pharmacies and to purchase online.
Are there any early symptoms of a tumor in the upper part of the lung? Is it possible to know if one is developing?
Tumors in the upper portion of the lung may have early symptoms that include pain in the upper back, shoulder blades, or arm because of the spread of cancer to the ribs, vertebrae, nerves, and blood vessels.
Respiratory symptoms, such as shortness of breath or a cough, do not typically occur with upper lung tumors. However, some people may have no symptoms at all, and others may have nonspecific symptoms, such as fatigue or loss of appetite.
A person should see a healthcare professional if they have any concerns.Elaine K. Luo, MD Answers represent the opinions of our medical experts. All content is strictly informational and should not be considered medical advice.