What is Pancoast Syndrome (Superior pulmonary sulcus tumors)?
Superior pulmonary sulcus tumors, also known as Pancoast tumors, are tumors that develop in the top part of your lung, just above your first rib. This area is also called the pleuro-pulmonary groove. When these tumors start to affect nearby structures like the network of nerves in your arm (brachial plexus), parts of your nervous system around your neck (cervical paravertebral sympathetic nervous system), and a group of nerve cells in your neck (stellate ganglion), it leads to a specific set of signs and symptoms known as Pancoast syndrome.
Pancoast syndrome can cause pain and a loss of sensation (paresthesias) in your shoulder and arm on the same side as the tumor, weakness (paresis), and loss of muscle in the base of your thumb (atrophy of the thenar muscles), as well as Horner’s syndrome, which involves droopy eyelids (ptosis), small pupils (miosis), and a lack of sweating (anhidrosis). However, it’s important to understand that Pancoast syndrome is different from a Pancoast tumor.
The most common cause of Pancoast syndrome is a type of lung cancer called primary bronchogenic carcinoma. The syndrome not only causes pain radiating to the shoulder blade area (parascapular pain), loss of muscle in the hand, but also shows up as a density at the top of the lung with localized damage to ribs and vertebrae in medical imaging tests.
What Causes Pancoast Syndrome (Superior pulmonary sulcus tumors)?
Pancoast syndrome is often caused by tumors in the upper part of the lung, generally a kind of lung cancer known as non-small cell lung cancer (NSCLC). NSCLC makes up approximately 80-85% of all lung cancers, while Pancoast tumors specifically account for about 3-5% of all lung cancers. It’s important to note that more than 95% of Pancoast tumors are NSCLC. There used to be a higher number of a type called squamous cell carcinoma, but recent studies suggest that now, adenocarcinoma is more common.
Besides originating from lung cells, Pancoast syndrome can be caused by other types of cancers such as adenoid cystic carcinomas (a rare type of cancer that can affect various body parts), thyroid cancers, lymphomas (cancers of the lymphatic system), or from metastasis, which is where cancer spreads from its original location. Even benign (non-cancerous) tumors in the upper part of the lung can cause Pancoast syndrome.
Less commonly, other cancers such as paravertebral thoracic schwannoma (a type of nerve tumor) and myxofibrosarcoma (a type of soft tissue tumor) can cause the symptoms associated with Pancoast syndrome. Lung infections or abscesses (a collection of pus) at the apex or top of the lungs can also lead to Pancoast syndrome affecting the chest wall and surrounding structures.
Risk Factors and Frequency for Pancoast Syndrome (Superior pulmonary sulcus tumors)
Lung cancer is the second most common type of cancer and is the leading cause of cancer-related deaths for both men and women in the United States and around the world. In 2017, over 200,000 people were diagnosed with lung cancer. Sadly, with a survival rate of only 18% after five years, more than 150,000 people lost their lives to this disease in the same year.
- Non-small cell lung cancer is the most prevalent type, making up 80% to 85% of all lung cancer cases.
- Pancoast tumors, on the other hand, only make up about 3% to 5% of all lung cancer cases.
Signs and Symptoms of Pancoast Syndrome (Superior pulmonary sulcus tumors)
Pancoast syndrome is a condition triggered by tumors that affect the grouping of nerves in your shoulder (brachial plexus) and can also cause discomfort in your chest wall, vertebrae, or ribs. The primary symptoms usually include pain in the shoulder or arm and a tingling sensation in the fourth and fifth fingers, the inner arm, and forearm. This is due to a problem with the nerve roots from C8 through T1. Because these tumors are located at the apex (top portion) of the lung, breathlessness is not a common initial symptom; it generally occurs later once the tumor begins to affect a larger portion of the lung.
Physical examination can reveal signs like facial flushing (turning red) and sweating on the same side as the tumor. These symptoms are due to the tumor affecting the sympathetic trunk (part of your nervous system) and cervical ganglion (a cluster of nerve cells in your neck). In advanced cases, the condition may also lead to Horner’s syndrome, which presents with drooping eyelids, constricted pupils, and lack of sweating.
- Pain in the shoulder or arm
- Tingling sensation in the fourth and fifth fingers, inner side of the hand, arm, and forearm
- Breathlessness (as the condition progresses)
- Facial flushing and sweating on the same side as the tumor
- Possible signs of Horner’s syndrome in advanced cases (drooping eyelids, constricted pupils, lack of sweating)
Testing for Pancoast Syndrome (Superior pulmonary sulcus tumors)
There are no special lab tests that directly point to a Pancoast tumor, a specific type of lung cancer.
At first, a chest X-ray might be done. This could show that the top area of the lung, called the apical cap, has grown in size. This could be a sign of a Pancoast tumor.
A CT scan, which is a type of X-ray that gives a more detailed image, can give more information about the size and location of the tumor, as well as whether there are other small tumors (satellite nodules) or enlarged lymph nodes (mediastinal adenopathy). All this information is important to know how advanced the tumor is. However, a CT scan is not so good at showing whether the tumor has spread to some areas, like certain nerves (brachial plexus), veins (subclavian vessels), and the chest wall. But it is good at showing whether the tumor has invaded bones.
After a Pancoast tumor has been diagnosed, an MRI scan of the neck, chest, and upper abdomen might be done before surgery. This scan is good at showing how far the tumor has spread, especially whether it has invaded nerves and blood vessels. Because of its detail, MRI is now the preferred method for scanning these tumors when surgery is being considered.
In order to definitely diagnose a Pancoast tumor, a piece of the tumor is often taken out with a needle in a process called a biopsy. This is typically guided by a CT scan, to make sure the needle gets to the right place. This method is usually successful in diagnosing a Pancoast tumor, with more than 90% success rate reported. In some cases, a bronchoscopy might be done, where a tube is inserted through the mouth into the lungs to examine them directly and collect samples. However, this method is only successful in diagnosing the tumor in about 30-40% of cases. Lastly, examining a sample of the patient’s spit (sputum cytology) or a biopsy of certain lymph nodes (scalene node biopsies) are usually not very helpful in diagnosing this type of tumor.
Treatment Options for Pancoast Syndrome (Superior pulmonary sulcus tumors)
The usual treatment plan for certain types of cancer involves a combination of chemotherapy and radiation therapy, followed by surgery to remove the remaining tumor.
Chemotherapy is a drug treatment that uses powerful chemicals to kill cancer cells. It often involves particular combinations of drugs, such as Cisplatin and Etoposide or Cisplatin, Mitomycin C, and Vindesine.
Radiation therapy, on the other hand, uses high-powered energy beams to kill cancer cells. Typically, patients receive around 27 doses over 5 to 6 weeks. After this ‘chemoradiation’ treatment phase, patients usually have surgery 4 to 6 weeks later to remove any remaining cancer.
However, not every patient is suitable for surgery. It may be ruled out if a patient has:
– The spread of cancer to other parts of the body, known as metastases
– Cancer cells present in lymph nodes in the chest or above the collarbone on the same or different sides as the primary cancer
– Cancer involving more than half of a backbone or vertebral body
– Cancer seeming to spread into the esophagus (the tube that moves food from your mouth to your stomach) and/or trachea (the main airway into the lungs)
– Cancer spreading to the uppermost part of the brachial plexus, a network of nerves that control the shoulder, arm, and hand.
Overall, the treatment journey involves a combination of therapies tailored for each patient’s unique situation.
What else can Pancoast Syndrome (Superior pulmonary sulcus tumors) be?
Pancoast syndrome can be caused by different types of health issues. It’s not just cancer that can lead to this syndrome, but benign, less harmful growths can also be a cause if they occupy a particular part of the lung called the ‘superior pulmonary sulcus’. The types of cancer that can lead to Pancoast syndrome include:
- Adenoid cystic carcinomas (a type of cancer in the glands)
- Hemangiopericytomas (a rare type of tumor)
- Thyroid carcinomas (cancer in the thyroid gland)
- Lymphomas (cancer of the immune system)
- Plasmacytomas (a type of bone marrow cancer)
- Any cancer that has spread from another part of the body
It’s also possible for certain infections at the top of the lung, or abscesses (pockets of pus), to cause Pancoast syndrome if they affect the chest wall and structures around it.
Surgical Treatment of Pancoast Syndrome (Superior pulmonary sulcus tumors)
If you have a type of lung cancer known as resectable non-small cell lung cancer (NSCLC) superior sulcus tumors, which affects a particular part of your lung, your doctor might recommend a combination of treatments. This might include chemotherapy drugs, radiation therapy, and surgical removal of the cancer. This combination is often recommended by the National Comprehensive Cancer Network, a team of cancer experts.
The idea is to first use chemotherapy and radiation together to shrink the tumor, this is called neoadjuvant chemotherapy. Then, they’ll remove what’s left of the tumor through surgery. After surgery, you’ll typically undergo more chemotherapy. With this approach, around half to almost three-quarters of patients are still alive after 2 years and nearly half are still alive after 5 years. Keep in mind though that exact survival rates can differ from person to person.
If your tumor is larger, or closer to your chest wall, airway, or mediastinum (the area in between your lungs), doctors usually perform surgery first. After this, they’ll determine the next steps based on how successful the surgery was. If they feel confident that they removed all of the tumor, you may only need chemotherapy. However, if there are cancer cells remaining at the edges of where the tumor was (known as positive surgical margins), you’ll likely have a combination of chemotherapy and radiation. Once that’s done, doctors might perform a second surgery to remove any remaining cancer, followed by more chemotherapy.
What to expect with Pancoast Syndrome (Superior pulmonary sulcus tumors)
Just like most types of cancer, individuals diagnosed with early-stage disease and in better health generally have a favourable outlook. On the other hand, advanced-stage disease, poor health, and weight loss often result in unfavorable outcomes. It’s also worth noting that patients with a particular type of lung cancer that has spread (metastatic NSCLC) who are suitable for targeted therapies usually have a better outlook than those who don’t have targetable genetic variations.
It’s crucial to quickly recognize and diagnose a condition known as Pancoast syndrome, a rare type of lung cancer that affects the top of the lung. Given that this condition is most often advanced (stage IIB or higher) by the time it’s diagnosed, early detection, accurate staging, and prompt treatment are vital for long-term survival.
Possible Complications When Diagnosed with Pancoast Syndrome (Superior pulmonary sulcus tumors)
The complications mostly stem from the treatments involved:
- Surgery: Among the possible side-effects are a condition called atelectasis (which is the partial or complete collapse of a lung), pain, abnormal changes in the shape (deformity) of the chest wall, frozen shoulder (which is stiffness, pain and limited movement in the shoulder), leakage of the fluid surrounding the brain and spinal cord, prolongation of air leaks and possible injury to a group of nerves in the neck and shoulder known as the brachial plexus.
- Chemotherapy: The treatment may cause various side effects due to the drugs involved.
- Radiation: Possible side effects include hair loss (alopecia), feeling sick in the stomach (nausea), vomiting, skin becoming tough and leathery, and impaired ability to heal wounds.
