Pancoast Syndrome – Causes, Symptoms, Treatment, Prognosis

What is Pancoast Syndrome?

Pancoast Syndrome is the name given to a type of lung tumor. It is a non-small cell tumor that spreads to encompass the nearby tissues, especially the ribs and the vertebrae. Pancoast Syndrome is also known by the name of superior sulcus tumor. As the tumor starts spreading it tend to cause a variety of neurological symptoms which are specific only to Pancoast tumors and thus the tumor and these specific symptoms together are termed as Pancoast Syndrome.[1,2,3]

Studies estimate that Pancoast tumors are quite rare and only constitute to about 5% of all cases. The only difference between Pancoast tumors and other forms of lung cancers is that these tumors grow on the top of the lungs and cause neurological symptoms, which is not the case in other tumors. Pancoast tumors begin as squamous cell cancer but at times can also begin as an adenocarcinoma. The structures that get affected by Pancoast tumors include the lymph vessels, nerves, ribs, and area in between the lungs.[1,2,3]

What Causes Pancoast Syndrome?

As stated, majority of the tumors seen with Pancoast Syndrome are non-small cell lung cancers and 95% of these tumors are present in the superior sulcus. In rare cases, Pancoast Syndrome can also be seen with small cell lung cancers as well. Even though non-small cell lung cancer is the primary cause for Pancoast Syndrome, the differential diagnosis is quite broad.[2]

This is because there are a host of medical conditions that can cause Pancoast Syndrome and this is the reason why an accurate histologic diagnosis is necessary for appropriate treatment for Pancoast Syndrome.[2]

What are the Symptoms of Pancoast Syndrome?

What makes Pancoast Syndrome different from other forms of lung cancer are the symptoms that the patient experiences. Since the tumor in most cases is present at the top of the lung, common symptoms like breathing problems or coughing are not seen with Pancoast Syndrome. The tumor instead exerts pressure on the nerves and because of this pressure the patient experiences severe pain in the upper half of the chest, neck, face, and even the arms.[3]

Some of the other symptoms seen with Pancoast Syndrome include constant pain in the shoulders, pain radiating from the chest to the arms, tingling and weakness in the hands and fingers. Some people also observe a change in the size of the arms where they tend to shrink because of the condition. As the tumor starts spreading the patient then will start to experience fatigue, loss of appetite, and symptoms that are commonly seen with other forms of cancer.[3]

A person with Pancoast Syndrome in most cases also develops Horner Syndrome. This is a condition which is caused when there is a disruption in the pathway of the nerves from the brain to the face causing a variety of neurological symptoms. Normally, only one side of the face is affected by this disorder. The pupil on the affected side will shrink in size. This is called as pinpoint pupil. There is also drooping of the eyelid on the affected side.[3]

How is Pancoast Syndrome Treated?

The overall health status, age, and the extent to which the tumor has spread determine the treatment approach for a person with Pancoast Syndrome. The treatment will be focused to relieve the symptoms and remove the tumor. Surgery, radiation and chemotherapy are the frontline protocols that are followed to treat Pancoast Syndrome.[3]

Additionally, the patient will be given pain medication to control the pain associated with this condition. Steroids will also be prescribed to relieve some of the pressure on the nerves exerted by the tumor. It should be noted that less than 50% of people with Pancoast tumor are not deemed to be a candidate for surgery. In many cases, surgery is not even an option as a result of the cancer spreading to various areas of the body.[3]

The surgery done for Pancoast Syndrome depends on the tissues that the tumor has damaged. In some cases, the entire diseased part of the lung may have to be removed along with adjoining tissues, and even the top of the ribs. In some instances, a major artery may have to be removed and replaced with an artificial one to maintain adequate blood supply. Surgery to treat Pancoast Syndrome is quite complicated with 5% mortality rate and 38% chances of complications.[3]

Some of the possible complications arising out of surgery to treat Pancoast Syndrome include infections, air embolism, neurological damage, and vascular complications.

These complications have been estimated by researchers after analyzing various case studies and doing a thorough research.[3]

Chemotherapy and Radiation Therapy is the most likely treatment offered for people with Pancoast Syndrome. Both of these treatments may be given in combination or separately.[3]

However, both of these treatment methods have significant side effects. Various studies have concluded that a combination of chemotherapy and radiation is the most effective way to treat Pancoast Syndrome even though the side effects may be quite severe with combination of these therapies. However, more research is being done to come up with novel ways of treating Pancoast Syndrome through biologic agents. Researchers are confident that these drugs will be far more effective than the existing treatments given for Pancoast Syndrome.[3]

What Is The Prognosis Of Pancoast Syndrome?

The overall prognosis for Pancoast Syndrome has improved a great deal over the past decade or two. This is because of the research and advances in medical technology over the years. As of now, the 2 year survival rate for a person with Pancoast Syndrome is about 55-70% which is quite good considering the aggressive nature of the tumor and the damage it causes.[3]

If surgery is successfully done and all the cancer cells are removed then the 5 year survival rate for a person with Pancoast Syndrome is about 55-77%. However, these numbers change depending on the stage of the cancer at the time of diagnosis. The prognosis is much better in people with stage 3 cancers than people with stage IV cancers.[3]

The prognosis is also quite poor for people who have Pancoast Syndrome along with Horner syndrome. Pancoast Syndrome is a treatable condition but there is always a chance of recurrence.[3]

In conclusion, Pancoast Syndrome is the name given to a tumor that develops at the top of the lungs. It is also known by the name of superior sulcus tumor. It is caused by non-small cell lung cancer. What makes Pancoast Syndrome different from other form of cancer are the symptoms that it causes. Unlike other cancers, Pancoast Syndrome causes neurological symptoms as the tumor puts pressure on the nerves.[1,2,3]

It is also quite common for people with Pancoast Syndrome to develop Horner syndrome which is again a nerve disorder with its own set of symptoms. A person with Pancoast Syndrome can be treated with radiation and chemotherapy, either in combination or separately and surgery, although not many people are eligible for it. Moreover, surgery for Pancoast Syndrome is quite complicated and may lead to undesirable complications.[1,2,3]

The prognosis of Pancoast Syndrome has improved over the last decade with about 70% survival rate for 2 years post diagnosis. The 5-year survival rate has also improved to around 70% depending on the stage at which the cancer was diagnosed. The condition is treatable but the recurrence rate is quite high for Pancoast Syndrome.[1,2,3]

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