Lung (pulmonary) sarcoma is a rare type of lung cancer. Most lung cancers start in epithelial cells that line your airways, but pulmonary sarcoma develops in connective tissue or other nonepithelial cells.

Lung sarcoma is more commonly referred to as pulmonary sarcoma. If it starts in the lungs, it’s classified as primary pulmonary sarcoma. If it spreads from other tissues into the lungs, it’s classified as metastatic pulmonary sarcoma.

Primary pulmonary sarcoma is a very rare but often aggressive type of cancer. Researchers estimate that these sarcomas account for 0.013% to 0.4% of cancerous lung tumors.

The most common treatment for pulmonary sarcoma is surgery. Chemotherapy is sometimes administered before surgery to shrink the tumors. That said, no standard treatment guidelines exist, due to the rarity of this condition.

Read on to learn more about pulmonary sarcoma, including symptoms, treatment options, and outlook.

The most common type of cancer is carcinoma, which develops in cells, called epithelial cells, that line your organs.

The two most common types of lung cancer are small lung cancer and non-small cell lung cancer. They are both carcinomas.

Sarcomas are tumors that develop in mesenchymal cells. These cells form tissues such as:

  • connective tissue
  • fat
  • blood vessels
  • nerves
  • bones
  • muscle
  • cartilage

Pulmonary sarcoma forms in the blood vessels and supportive tissue in your lungs. It tends to be aggressive, with poor survival rates, though it’s important to note that most studies examining its outcomes have small sample sizes.

Most pulmonary sarcomas are metastatic. This means they spread from other tissues. About 40% of metastatic sarcomas spread to the lungs.

Symptoms of pulmonary sarcoma tend to be general and can mimic symptoms of many other conditions or other types of lung cancer. Symptoms might not appear until the cancer reaches the late stages.

They might include:

Researchers don’t exactly know what causes pulmonary sarcoma. Like other cancers, it develops when DNA changes cause cells to replicate (create copies) out of control.

For primary pulmonary sarcomas, this replication of cells creates a tumor in your lungs. For metastatic pulmonary sarcoma, the tumors start in other parts of your body. The cancer cells can then travel through your blood or lymph system to your lungs.

In a 2020 case series of 26 people from Greece who had primary pulmonary sarcoma, the average age at the time of diagnosis was 62 years, and the age range of people was 31 years to 75 years.

Receiving a lung cancer diagnosis often starts by visiting your primary healthcare professional. They’ll perform initial tests such as:

  • reviewing your personal and family medical history
  • asking about your symptoms and when they started
  • performing a physical exam where they may:
    • listen to your breathing
    • feel your chest
    • check you for signs of an infection, such as fever

If they suspect that you may have cancer, they will likely refer you for more tests. You might receive imaging tests such as:

If imaging suggests that you might have lung cancer, you may need a test called bronchial fibroscopy. This test involves inserting a special tube through your airways to examine your lungs and to take a sample of tissue for lab testing. This sample is called a biopsy.

At what stage is pulmonary sarcoma typically discovered?

Pulmonary sarcoma is often advanced at the time of diagnosis. In a 2021 review of 100 people with pulmonary sarcoma or pulmonary carcinosarcoma, nearly half of the people with pulmonary sarcoma were in lung cancer stage 3A when they were diagnosed.

Stage 3A can mean the tumor is:

  • between 1.2 inches (in) and 2 in, or between 3 centimeters (cm) and 5 cm
  • smaller than 2 in and has grown into the main airway of the lung
  • smaller than 2 in and has spread to the membrane covering your lung
  • smaller than 2 in and has caused the lung to partly or completely collapse
  • in surrounding lymph nodes, the area where the windpipe divides, or the space between the lungs
  • not yet in distant body parts

Due to the rarity of pulmonary sarcoma, no specific guidelines have been established for treating it.

The most common treatment is surgery to remove the tumor. Sometimes, a doctor will recommend undergoing chemotherapy first to shrink the tumor before surgery.

In a 2021 study including 100 people receiving treatment for primary pulmonary sarcoma from 1998 to 2019, more than two-thirds of people had surgery.

Types of surgery that might be performed include:

  • lobectomy to remove one lobe of your lung
  • pneumonectomy to remove an entire lung
  • procedures to remove a small section of your lung such as:
    • wedge resection
    • sleeve resection
    • segmentectomy
  • lymph node removal to remove surrounding lymph nodes

Learn more about lung cancer surgery.

In the same 2021 study reviewing 100 people, researchers reported a median survival of 39.6 months for people with primary pulmonary sarcoma who had surgery and 4.9 months for people who didn’t have surgery.

Factors linked to improved survival were:

  • surgery
  • younger age
  • nonsmoker
  • certain microscopic features of the cancer

Pulmonary sarcoma is a rare type of cancer that develops in the supportive tissue or blood vessels in your lungs. Most cases spread to your lungs from other body parts.

The outlook for pulmonary sarcoma is often poor, and there are no standard treatment guidelines due to its rarity. Surgery and chemotherapy are among the most common treatments.