Lung (pulmonary) sarcoma is a rare type of cancer that forms in the blood vessels and supportive tissue in your lungs.

Most lung cancers, including small cell and non small cell lung cancers, are carcinomas. This means the cancer develops in the epithelial cells that line your airways.

Pulmonary sarcoma, however, develops in non-epithelial cells, such as mesenchymal cells. These cells form connective tissues, including:

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

Pulmonary sarcoma tends to be an aggressive type of lung cancer, but it’s important to note that most studies examining its outcomes have small sample sizes.

A healthcare professional can help develop an appropriate treatment plan to help manage symptoms and, if possible, shrink the tumors.

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

Types of pulmonary sarcoma

Healthcare professionals typically classify lung sarcoma into two categories:

  • primary, which starts in the lungs
  • metastatic, which spreads from other tissues into the lungs

Most pulmonary sarcomas are metastatic. About 40% of metastatic sarcomas spread to the lungs.

Primary pulmonary sarcoma is very rare. Researchers estimate that these sarcomas account for 0.013% to 0.4% of cancerous lung tumors.

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.

In primary pulmonary sarcomas, this cell replication leads to a tumor in your lungs. For metastatic pulmonary sarcoma, the tumors start in other parts of your body, and the cancer cells 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 the people was 31 to 75 years.

A lung cancer diagnosis often starts with a visit to a healthcare professional, such as a primary care doctor. 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 for signs of infection, such as fever

If they suspect that you may have lung cancer, they will likely refer you to a lung specialist, known as a pulmonologist, 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 into your airways to examine your lungs and obtain a tissue sample 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 the 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 of 100 people receiving treatment for primary pulmonary sarcoma from 1998 to 2019, more than two-thirds underwent surgery.

Types of lung 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 the surrounding lymph nodes

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

Factors linked to improved survival were:

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

It’s important to note that life expectancy varies from person to person. A healthcare professional can help you better understand your outlook based on your condition.

In some cases, surgeons can remove sarcomas, according to Cancer Research UK. But this doesn’t mean it is cured. In most cases, lung sarcoma is diagnosed in the late stages, so treatment typically aims to slow disease progression and manage symptoms.

Sarcomas most commonly metastasize to the lungs, according to Cancer Research UK.

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 less favorable, and there are no standard treatment guidelines due to its rarity. Surgery and chemotherapy are among the most common treatments.