What Is a Pancoast Tumor?

How are Pancoast Tumors Different From Other Lung Cancers?

Young man applying ointment to his shoulder
What are pancoast tumors and how do they differ from other lung cancers?. Stockbyte/Stockbyte/Getty Images

What are Pancoast tumors and how do they differ from other types of lung cancer as far as symptoms, and treatment?

Pancoast Tumors - Definition

Pancoast tumors are lung cancers that begin at the top of the right or left lung and invade the chest wall. They are also called superior sulcus tumors. Pancoast tumors often come along with unique symptoms known as “Pancoast syndrome,” which consists of pain in the shoulder and the inside of the arm and hand.

 Pancoast tumors differ from other types of lung cancer in many important ways.

Symptoms of Pancoast Tumors

Symptoms of a Pancoast tumor are due to compression of structures (nerves) that lie near the upper part of the lung where the cancer is present. The classic symptoms are called the Pancoast-Tobias syndrome and include:

  • Arm and shoulder pain frequently radiating down the arm (especially the inside part of the arm and on the pinky side of the hand.)  This pain is often severe and unrelenting.  Pain can also radiate into the armpit or into the shoulder blade.
  • Weakness in hand muscles. 
  • Tingling and prickly sensations in the hand, especially the ring and pinky fingers.
  • "Horner's syndrome" which includes ptosis (a droopy eyelid) anhidrosis (an eventual lack of sweating on one side of the face that is often preceded by flushing and sweating on one side of the face) and miosis (a constricted pupil of the eye.)

    Sometimes there is also swelling of the upper arm due to pressure on the subclavian vein. If you have any symptoms suggestive of a Pancoast tumor, talk to your doctor or get a second opinion.  Many of these tumors are missed at first due to both their unique symptoms and because they can be difficult to see on x-rays.

    Location and Anatomy of Pancoast Tumors

    Pancoast tumors occur in the upper part of the right or left lung (the apical region) and invade structures near this area. These can include:

    • Ribs (often the 1st and 2nd ribs) and vertebrae.
    • The brachial plexus – a cluster of nerves located near the armpit.
    • Nerves or blood vessels near the top of the lungs (for example, the subclavian artery, the phrenic nerve, the recurrent laryngeal nerve, and the vagus nerve.)

    Causes of Pancoast Tumors

    Smoking is responsible for many of these cancers, but it's important to note that the diagnosis of lung cancer is currently more common in former smokers than in people who currently smoke.  Other risk factors include radon exposure, secondhand smoke, and occupational exposures to chemicals and substances known to cause lung cancer.

    Diagnosis

    Diagnosis of Pancoast tumors is frequently delayed for two reasons. These tumors are less likely to have typical lung cancer symptoms, such as shortness of breath and coughing, and people often first see an orthopedic surgeon or neurologist for their symptoms.

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

    A combination of CT scans and MRI's are often used - with MRI important before surgery to look for nerve involvement.  A biopsy of some form - whether an open chest biopsy (thoracotomy) or through biopsy of the lymph nodes above the collar bone (supraclavicular biopsy) is often done to make the diagnosis.  Other tests, such as a bronchoscopy may be done as well.

    Staging

    Staging is often done with a combination PET scan/CT.  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.

    Type of Lung Cancer

    Pancoast tumors are usually a form of non-small cell lung cancer.  Of these, around 2/3 are lung adenocarcinoma and 1/3 are squamous cell carcinoma or large cell carcinoma.  Since there are several targeted therapies now available for lung adenocarcinoma, and now for squamous cell carcinoma as well, it is very important to make sure you have gene profiling (molecular profiling) done on your tumor.

    Treatment

    Chemotherapy and targeted therapies

    Treatment for a Pancoast tumor usually begins with lung cancer chemotherapy, whether or not surgery would be possible.  Due to the location which can make surgery difficult, the goal is to reduce the tumor in size as much as possible before surgery.  In addition, many of these tumors have "targetable mutations" - genetic abnormalities found on gene testing for which targeted therapies are available.  If you have not had gene testing, also called molecular profiling - done on your tumor, talk to your doctor.

    Surgery

    Surgery can often be done on Pancoast tumors, but is usually done after chemotherapy and/or targeted therapy to shrink the tumors.  Chemotherapy before surgery is called "induction therapy."  This surgery can be very difficult, and it's important to find a cancer center in which the surgeons are familiar with this type of tumor.  Unlike some cancers which have spread to bones, some people with Pancoast tumors which has invaded the vertebrae can be successfully treated with surgery.  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 a palliative therapy -- one used to relieve pain and other symptoms. 

    Targeted therapy

    As noted above, everyone with non-small cell lung cancer and especially lung adenocarcinoma should have molecular profiling done on their tumor.  Medications are currently approved for those with EGFR mutations, ALK rearrangements, and ROS1 rearrangements, with further treatments being studied in clinical trials.

    Immunotherapy

    Immunotherapy is an exciting new way to treat lung cancer, with the first treatments approved in this category in 2015.  While it does not work for everyone, some people - even those with advanced lung cancer - have had long-term control of their disease with these treatments.

    Clinical trials

    Pancoast tumors are quite rare, and clinical trials are in progress to evaluate new treatments.

    Prognosis of Pancoast Tumors

    A significant improvement in survival from Pancoast tumors has been achieved in the past few decades. In general, Pancoast tumors have a better prognosis than tumors that are located more centrally in the lungs, and the survival rate may be better than other cancers at a similar stage

    The 2-year survival rate was found to be between 55 percent and 70 percent - significantly higher than some other lung cancers, and of those who have Pancoast tumors which could be treated with surgery, the 5-year survival rate was between 50 and 77%.

    It's important to note that these survival rates could very well be higher now, since newer targeted therapies, as well as an immunotherapy drug, have been approved since these studies were completed.

    If You Have Been Diagnosed with a Pancoast Tumor

    Since Pancoast tumors are fairly uncommon, and since surgery is tricky, it is very important to consider getting a second opinion if you've been diagnosed with a Pancoast tumor.  Studies tell us that learning as much as possible about your cancer not only helps you feel more in control but may help with your outcome as well.  Check out these tips on what to do when you are newly diagnosed, as well as how to research your cancer online.

    Being your own advocate in your cancer care has made a difference for many people, and according to the National Cancer Institute, people with lung cancer should consider the option of clinical trials.

    You may have heard more about breast cancer and pink ribbons, but the lung cancer community is active and very supportive.  Even if you aren't the type of person who likes support groups or social media, consider connecting with others living with the same disease.

    And, if it is your loved one who has been diagnosed instead, check out these thoughts on when your loved one has lung cancer.

    Sources:

    Caronia, F., Fiorelli, A., Ruffini, E. et al. A comparative analysis of Pancoast tumour resection performed via video-assisted thoracic surgery versus standard open approaches. Interactive Cardiovascular and Thoracic Surgery. 2014. 19(3):426-35.

    Deslauriers, J., Tronc, F., and D Fortin. Management of tumors involving the chest wall including Pancoast tumors and tumors invading the spine. Thoracic Surgery Clinics. 2013. 23(3):313-25.

    Foroulis, C., Zaraogoulidis, P., Darwiche, K. et al. Superior sulcus (Pancoast) tumors: current evidence on diagnosis and radical treatment. Journal of Thoracic Disease. 2013. 5 Suppl 4:S342-58.

    Nikolaos, P., Vasilios, L., Efstratios, K. et al. Therapeutic modalities for Pancoast tumors. Journal of Thoracic Disease. 2014. 6 Suppl 1:S180-93.

    Ozmen, O., Yilmaz, Ul, Dadali, Y. et al. Use of FDG PET/CT in Patients with Pancoast Tumors: Does it Add Any Contribution to Patient Management?. Cancer Biotherapy & Radiopharmaceuticals. 2015. 30(8):359-67.

    Panagopoulos, N., Leivaditis, V., Koletsis, E. et al. Pancoast tumors: characteristics and preoperative assessment. Journal of Thoracic Disease. 6 Suppl 1:S108-15.

    White, H., White, B., Boethel, C., and A. Arroliga. Pancoast’s syndrome secondary to infectious etiologies: a not so uncommon occurrence. American Journal of the Medical Sciences. 2011. 341(4):333-6.

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