Pancoast’s Tumour

Pancoast’s tumour is a rare type of lung cancer that develops in the top part of the lung and causes unusual symptoms like severe shoulder pain and arm weakness rather than typical lung cancer signs such as coughing or breathing problems.

Table of contents

• What is Pancoast’s tumour?
• Other names
• Associated anatomy
• How common is this condition?
• Types of Pancoast’s tumours
• Signs and symptoms
• Causes and risk factors
• How the tumour spreads
• Diagnosis
• Treatment options

What is Pancoast’s tumour?

A Pancoast tumour is a rare type of lung cancer that begins in the upper part of your lung, above the first rib^[1]. The cancer develops in an area called the apex, which is the very top of the lung^[3].

What makes this cancer different from other lung cancers is its location. Because it grows at the top of the lung, it typically spreads into nearby structures rather than staying within the lung tissue itself. The tumour can invade the ribs, vertebrae (bones in the spine), nerves that control the shoulder and arm, and blood vessels^[4].

When a Pancoast tumour presses on a bundle of nerves called the brachial plexus (which runs from the upper chest into the neck and arms), it creates a specific pattern of symptoms. Doctors call this collection of symptoms Pancoast syndrome or Pancoast-Tobias syndrome^[1][3].

The tumour was named after Henry Pancoast, an American radiologist who first described these cancers in detail in 1924 and 1932^[6].

superior sulcus tumor, superior pulmonary sulcus tumor

Other names

Pancoast’s tumour is also known as a superior sulcus tumour or superior pulmonary sulcus tumour^[4][6]. The superior sulcus is an anatomical groove in the top part of the lung formed by blood vessels and other structures^[3].

Associated anatomy

• Lung apex (top part of the lung)
• First and second ribs (thoracic ribs)
• Brachial plexus (bundle of nerves)
• Subclavian artery and vein (blood vessels)
• Sympathetic nerve chain and stellate ganglion
• Upper vertebrae (spine bones)
• Upper chest wall and back

How common is this condition?

Pancoast tumours are rare. They make up less than 5% of all lung cancer cases^[1][3]. According to research, they account for approximately 3 to 5% of lung cancers^[3][4].

These tumours most commonly occur in people in their 60s or 70s, and they are seen more often in men than in women^[3][6].

Types of Pancoast’s tumours

Most Pancoast tumours are a type called non-small cell lung cancer. More than 95% of all Pancoast tumours fall into this category^[3].

The most common subtype is adenocarcinoma, which makes up about 60% of cases^[14]. These cancers begin in the mucus-making gland cells in the lining of the airways^[1][4].

Other types include squamous cell carcinoma and large cell carcinomas. Small cell lung cancers can also occur as Pancoast tumours, but these are very rare^[1][8].

Signs and symptoms

Unlike typical lung cancers, Pancoast tumours do not usually cause coughing or chest pain in the early stages. Instead, the most common first symptom is severe shoulder pain, which occurs in up to 96% of patients^[6][1].

The symptoms appear only on the side of the body where the tumour is located. For example, if the tumour is in the right lung, you will have symptoms on the right side of your body^[1].

Common symptoms of Pancoast syndrome include:

• Severe pain in the shoulder or shoulder blade
• Pain that travels down the arm to the wrist, particularly along the inner side of the arm toward the pinky finger
• Weakness in the arm and hand
• Neck pain and upper back pain
• Loss of ability to use the hand and fingers properly
• Tingling or numbness in the hand
• Pain in the upper ribs
• Swelling in the upper arm

General cancer symptoms may also be present, including tiredness, unexplained weight loss, tightness in the chest, and feeling generally unwell^[1][6].

Between 15% and 50% of people with Pancoast tumours develop a condition called Horner’s syndrome. This happens when the tumour affects the sympathetic nerves that run to the head^[3][6]. Horner’s syndrome causes:

• A droopy eyelid on the affected side
• A smaller pupil in the affected eye
• Lack of sweating on one side of the face
• Flushing on one side of the face

Because these symptoms are unusual and do not suggest lung cancer at first, Pancoast tumours are often initially mistaken for other conditions such as shoulder injuries, frozen shoulder, arthritis, or problems with the neck bones^[5][6]. This can lead to delays in diagnosis.

Causes and risk factors

The causes and risk factors for Pancoast tumours are similar to those for other types of lung cancer. The main risk factors include^[1]:

• Smoking tobacco
• Exposure to secondhand smoke
• Long-term exposure to harmful substances such as asbestos, radon gas, and heavy metals like nickel or gold

While most Pancoast tumours are cancers that start in the lung, in very rare cases, other diseases can cause similar symptoms. These include cancers that have spread from other parts of the body (such as thyroid cancer or lymphoma) and certain infections^[3].

How the tumour spreads

Pancoast tumours tend to invade nearby structures early in their development because of their location at the top of the lung^[8]. The tumour typically spreads directly into surrounding tissues rather than traveling through the bloodstream or lymphatic system in the early stages.

Most commonly, Pancoast tumours spread into^[1]:

• The upper chest wall
• The upper back
• The first and second ribs
• Blood vessels that supply the arms
• Nerves that control the arm and hand

In more advanced cases, the cancer may spread to lymph nodes or distant parts of the body^[4].

Diagnosis

Diagnosing a Pancoast tumour can be challenging. In the early stages, these tumours often do not show up clearly on regular chest X-rays because of their location at the very top of the lung^[1][4].

If your doctor suspects a Pancoast tumour based on your symptoms, they will order additional tests. These may include^[1][4]:

Imaging tests create detailed pictures of the inside of your body:

• CT scan (computed tomography): Uses X-rays and computers to create detailed cross-sectional images of the chest
• MRI scan (magnetic resonance imaging): Uses magnets and radio waves to create detailed images, particularly helpful for seeing how far the tumour has spread into nerves and blood vessels
• PET-CT scan (positron emission tomography): Uses radioactive sugar to identify cancer cells, as cancer cells absorb more of this sugar than normal cells

Biopsy involves taking a small sample of tissue from the tumour to examine under a microscope. This confirms whether the tumour is cancer and what type it is. Different methods can be used^[4]:

• Needle biopsy: A thin needle is inserted through the skin to collect tissue
• Bronchoscopy: A thin tube with a camera is passed down the throat into the airways
• Video-assisted thoracoscopy or small surgical opening: Used when other methods cannot reach the tumour

Other tests may include endoscopic ultrasonography, which uses sound waves to create images and help locate the exact position of the tumour^[5].

Treatment options

Treatment for Pancoast tumours depends on the stage of the cancer, its exact location, whether it has spread to nearby structures or lymph nodes, and your overall health^[4][10].

The standard treatment approach uses three different methods together, which doctors call trimodal therapy^[11][14]:

Step 1: Chemotherapy and radiation therapy before surgery

If you are healthy enough for surgery, you will usually receive a combination of chemotherapy (cancer-killing drugs) and radiation therapy (high-energy rays that destroy cancer cells) before the operation. This is called neoadjuvant treatment or preoperative chemoradiotherapy^[4][10].

The purpose of this treatment is to shrink the tumour and kill any cancer cells that may have started to spread. This makes surgery safer and more likely to be successful. The treatment typically lasts several weeks^[4][12].

Step 2: Surgery

After a waiting period of 2 to 4 weeks following chemoradiotherapy, surgery is performed to remove the tumour^[10]. Surgery for Pancoast tumours is complex and must be done by specialist surgeons at specialist cancer centers^[4].

The operation usually involves removing the affected part of the lung along with the top two ribs and sometimes additional ribs. In some cases, the surgeon may need to remove a major blood vessel and replace it with an artificial tube to maintain blood supply to the arm^[4][12].

Step 3: Treatment after surgery

After surgery, additional chemotherapy or other treatments may be recommended to destroy any remaining cancer cells^[10].

If surgery is not possible

Some patients cannot have surgery because the tumour has invaded critical structures that cannot be removed safely, or because the cancer has spread too far. In these cases, treatment typically involves^[4][10]:

• Chemoradiotherapy: Combined chemotherapy and radiation therapy to shrink the tumour and control symptoms
• Immunotherapy: Medicines that help the immune system recognize and attack cancer cells, which may be given after chemoradiotherapy
• Targeted therapy: If your cancer cells have certain specific changes, you may be offered targeted drugs that attack those specific features
• Radiation therapy alone: To shrink the tumour and reduce symptoms

For patients experiencing severe ongoing pain, additional procedures may be offered to help control pain and improve quality of life^[10].

Some patients cannot have surgery because of other health conditions such as heart disease, recent heart attack, or poor lung function. These factors are carefully evaluated before treatment decisions are made^[10].