Pulmonary lymphoma is a rare cancer in which lymphoma cells affect your lungs. It can start inside the lungs (primary) or elsewhere in the body and spread to the lungs (secondary).
Lymphoma occurs when lymphocytes, a type of white blood cell, start to grow out of control. It affects the lymphatic system, a network of vessels, organs, and tissues throughout your body where lymphocytes are created and stored.
Pulmonary lymphoma occurs when lymphoma affects your lungs. While lung cancer is the most common cancer and the leading cause of cancer death worldwide, pulmonary lymphoma is rare. Experts think that’s because there’s not a lot of lymph tissue in the lungs.
Pulmonary lymphoma tends to have a more favorable outcome than other lung cancers. Still, your specific outlook will depend on the type of lymphoma, the stage of the cancer, and other factors, like your age and overall health.
Doctors can classify pulmonary lymphoma as either primary or secondary.
Primary pulmonary lymphoma
Primary pulmonary lymphoma (PPL) means the cancer starts inside and is contained within the lungs. PPL is rare, accounting for 0.5% of all primary lung cancers and less than 1% of all lymphomas.
PPLs can be either Hodgkin’s (HL) or non-Hodgkin’s lymphoma (NHL), though the latter is more common.
Most PPLs (70% to 90%) are a subtype of NHL called mucosa-associated lymphoid tissue (MALT) lymphoma. Another common type is diffuse large B-cell lymphoma (DLBCL). Several rarer types are also possible.
Secondary pulmonary lymphoma
Secondary primary lymphoma (SPL) means the lymphoma started elsewhere in the body and later spread to the lungs. SPL is more common than PPL.
The most common types of SPL are:
Pulmonary lymphoma doesn’t always cause symptoms. When symptoms are present, they may include:
Pulmonary lymphoma is rare, and its symptoms are similar to those of many other conditions. For this reason, obtaining a
A doctor will evaluate your risk factors for pulmonary lymphoma, your symptoms, and your medical history.
Diagnostic tests may include:
Treatment options for pulmonary lymphoma include:
According to a 2022 case series, surgery and radiation may be options for people with limited tumor spread in the lungs. Chemotherapy is a better first-line treatment for people with more diffuse tumors.
Some types of pulmonary lymphoma grow very slowly. In this case, a doctor may recommend not treating it or waiting until it shows evidence of growth before recommending treatment. This is called watchful waiting or active surveillance.
Doctors typically treat SPL with the same regimen they use to manage the primary cancer. This usually involves a combination of radiation, chemotherapy, and targeted treatments.
Your specific treatment plan for SPL may require adjustments based on previous treatments your care team has tried for the primary cancer.
All cancers are caused by errors (mutations) that occur in the DNA of your cells. The mutations cause these cells to divide out of control and form tumors.
In PPL, these mutations affect cells in your lungs.
Researchers don’t fully understand what causes these mutations, but they suspect genetics, environmental factors, certain infections, and diseases that affect the immune system may play a role.
SPL occurs when lymphoma spreads to your lungs from another part of the body. The lymphoma cells
Pulmonary lymphoma usually affects older adults but can affect anyone. In a 2019 single-center study, the median age of diagnosis was 55 years, but participants ranged in age from 15 to 83 years. While this cancer rarely affects people under age 30 years, there are rare cases of
You may have a higher risk of pulmonary lymphoma if you have an autoimmune disease, like:
Other risk factors for pulmonary lymphoma include:
- HIV infection
- solid organ transplantation
- immunodeficiency
PPL tends to be less aggressive and less likely to spread, so people with PPL may have a more favorable outlook than those with other types of cancers.
In a 2019 study, for example, the 5-year survival rate for 80 people with MALT lymphoma (the most common form of PPL) was
Your outlook will depend on many factors, including the type and stage of lymphoma, your age, and your overall health.
People with pulmonary lymphoma secondary to NHL or HL may have a shorter survival rate compared with those who did not develop secondary lung cancer. Early detection of lung tumors can improve your outlook.
Pulmonary lymphoma is a rare cancer that forms in the lungs. In most cases, lymphoma has spread to the lungs after originating elsewhere (SPL). Rarely, lymphoma may start in lung tissue (PPL).
Diagnosis can be challenging as its symptoms, like cough, fever, and chest pain, can resemble many other conditions. Consider talking with a doctor if you experience lung symptoms that don’t go away and have any risk factors for pulmonary lymphoma, like an autoimmune disease.
PPL treatment usually involves surgery, chemotherapy, and radiation, and the outlook is usually more favorable than for other types of lung cancer. Your treatment and outlook with SPL will depend on factors specific to the primary cancer site.