What Is a Pancoast Tumor?

Symptoms, diagnosis, treatment, and prognosis of this type of lung cancer

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Pancoast tumors are a type of lung cancer that invades the chest wall. These tumors are defined by their location and are sometimes referred to as superior sulcus tumors. They occur in the upper part of the lung (the apical region) and invade structures near this area including:

  • Ribs (often the first and second ribs) and vertebrae
  • The brachial plexus (a cluster of nerves located near the armpit)
  • Nerves or blood vessels near the top of the lungs (including the subclavian artery, the phrenic nerve, the recurrent laryngeal nerve, and the vagus nerve)

Pancoast tumors account for just 3% to 5% of lung cancers, and their diagnosis is often delayed. Symptoms can mimic those of bone and joint diseases, and tumors can be difficult to spot on X-rays. However, many are diagnosed at a stage when surgery can be done (often after chemotherapy and radiation).

pancoast tumors
Verywell / Madelyn Goodnight

Types of Pancoast Tumors

Pancoast tumors are a form of non-small cell lung cancer. The most common type is lung adenocarcinoma, followed by squamous cell carcinoma.

Symptoms

Symptoms of a pancoast tumor are caused by compression of structures (nerves) that lie near the upper part of the lung where the cancer is present. The tumor is associated with a unique set of symptoms known as “Pancoast syndrome." These symptoms include:

Arm and Shoulder Pain

Pain in the shoulder is the most common early symptom of a Pancoast tumor. Pain frequently radiates down the arm (especially on the inside of the arm and on the pinky side of the hand) and is often severe and unrelenting. Pain can also radiate into the armpit or to the shoulder blade.

Weakness in Hand Muscles

People may feel weakness in the muscles of the hand that can easily be dismissed as musculoskeletal in origin.

Tingling or Numbness in the Hand

Tingling or numbness is especially common in the ring and pinky fingers.

Horner's Syndrome

Horner's syndrome is a collection of symptoms that occur only on one side of the face. These include:

  • Drooping upper eyelid
  • Diminished or complete absence of sweating
  • Inward sinking of the eyeball
  • Smallness or excessive constriction of the pupil of the eye

Swelling of the Upper Arm

Sometimes there is also swelling of the upper arm due to pressure on the subclavian vein (the vein that runs beneath the collarbone).

Causes

Smoking is a primary risk factor in lung cancers, and cases have decreased overall due to declining numbers of people who smoke. However, it's important to note that the diagnosis of lung cancer is growing in non-smokers (either former smokers or those who have never smoked, and especially in young women).

Other risk factors for lung cancer include radon exposure, secondhand smoke, and occupational exposures to chemicals and substances known to cause lung cancer.

Diagnosis

The average age of Pancoast tumor diagnosis is around 60 years, and men are more frequently affected than women.

Diagnosis of Pancoast tumors is frequently delayed. One reason is that these tumors are less likely to have typical lung cancer symptoms (such as shortness of breath and coughing), so symptoms may be mistaken for a neurological or orthopedic condition.

Pancoast tumors are also difficult to see on chest X-rays due to their location.

As a result, once these tumors are accurately diagnosed, they are usually at an advanced stage.

A combination of CT and MRI scans is often used. An MRI is important before surgery to look for nerve involvement. A lung biopsy of some type—whether an open chest biopsy (thoracotomy) or a biopsy of the lymph nodes above the collarbone (supraclavicular lymph node biopsy)—is often done to make the diagnosis. Other tests, such as a bronchoscopy may be done as well.

Staging

Pancoast tumors are staged similarly to other lung cancers, by using Roman numerals I to IV and the subtypes A or B to indicate the progression of the disease. Many of these cancers are stage IIB non-small cell lung cancer.

Based on TNM lung cancer staging, most of them are T3 or T4. This means that the tumor is often larger than 7 centimeters (or 3.5 inches) in diameter at the time of diagnosis.

Staging is often done with a combination PET scan/CT. A PET scan be very important in planning treatment, as a 2015 study found: PET scans changed the staging (and consequently the best treatment choices) for 21% of people with this type of tumor.

Differential Diagnosis

While Pancoast tumor symptoms are most often associated with lung cancer, they sometimes occur with other conditions, such as other cancers, bacterial or fungal infections, or diseases like tuberculosis.

Treatment

Treatment options for people with Pancoast tumors depend upon the extent of the tumor. Currently, the preferred treatment includes a combination of chemotherapy and radiation therapy followed by surgery. Options include:

Chemotherapy

Treatment for a Pancoast tumor usually begins with lung cancer chemotherapy, whether or not surgery is possible immediately. Due to the location of this type of tumor, which can make surgery difficult, the goal is to reduce its size as much as possible before surgery. Chemotherapy before surgery is called "induction therapy."

Targeted Therapies

Many Pancoast tumors have targetable mutations. These are genetic abnormalities found through gene testing for which targeted therapies are available. Targeted therapies are a form of precision medicine selected for based on precise information about your specific disease.

Since several targeted therapies are now available for lung adenocarcinoma and for squamous cell carcinoma, it is very important to make sure you have gene profiling (molecular profiling) done.

Medications are currently approved for those with EGFR mutations, ALK rearrangements, ROS1 rearrangements, BRAF mutations, and more, with further treatments being studied in clinical trials.

Surgery

Surgery can often be done on Pancoast tumors. It is usually performed after chemotherapy and/or targeted therapy plus radiation therapy to shrink the size of tumors. Because this surgery can be challenging, it's important to find an experienced surgeon familiar with this condition.

While surgery for Pancoast tumors has generally been done via a thoracotomy (open chest surgery in which a rib is separated or removed), a 2014 study suggested that minimally invasive surgery with video-assisted thoracoscopic surgery (VATS) may be safe and effective. VATS involves making small incisions in the chest and using instruments to remove a tumor.

Since Pancoast tumors are often located close to nerves, it's recommended that both a thoracic surgeon and a neurosurgeon be present for these surgeries. During surgery, lymph nodes in the chest between the lungs (called mediastinal lymph nodes) are often removed as well.

Radiation Therapy

If treatment aimed at a cure is not possible, radiation therapy can still be helpful as palliative therapy to relieve pain and alleviate other symptoms. Radiation therapy may also be used along with chemotherapy to "shrink" a tumor before surgery.

Immunotherapy

Immunotherapy is a possible treatment for some non-small cell lung cancers. This treatment uses your body's own immune system to attack cancer.

There are “checkpoint” proteins on immune cells that act like switches that need to be activated to start an immune response. Cancer cells can de-activate these checkpoints to avoid being attacked by the immune system. Drugs that target these proteins are called checkpoint inhibitors, and they allow the immune system to attack the cancer.

Some immunotherapy drugs approved to treat Pancoast tumors include:

  • Opdivo (nivolumab)
  • Keytruda (pembrolizumab)
  • Libtayo (cemiplimab) 
  • Tecentriq (atezolizumab)

Prognosis

Survival rates for Pancoast tumor have been improving, and depend largely on when the tumor is diagnosed, if it has metastasized to lymph nodes or other organs, and how effective treatments were.

For patients with surgically treatable cancer, the five-year survival rate is generally considered to be between 30% and 50%, although significantly higher rates have been reported.

The five-year survival rate tends to be lower in patients with late-stage inoperable tumors that have invaded the lymph nodes.

A Word From Verywell

Being diagnosed with cancer can be difficult to accept. Seeking out a support group or a mental health counselor can help you cope with your fears and emotions and help you feel less alone. Your healthcare provider can help you find a therapist and/or group specifically geared to cancer patients, as can organizations like the American Cancer Society (ACS).

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