Pleuropulmonary Blastoma: Causes, Symptoms, and Treatment
Medically reviewed by Harsha Moole, MD, Internal Medicine. Last reviewed: October 2, 2026.
What kind of doctor treats Pleuropulmonary Blastoma?
A pulmonologist, a doctor who specializes in lung and breathing problems, is often involved in evaluating chest growths. Children are usually treated by a team that also includes a pediatric oncologist, who treats cancer in children, along with surgeons and other specialists.
What it is
Pleuropulmonary blastoma is a rare cancer that starts in the chest, usually in the lung or the tissue lining the lung and chest wall. It grows mostly in babies and young children and is very uncommon in adults. Doctors group it into types based on how it looks under a microscope.
Symptoms
Many children have no symptoms at first, and the growth is sometimes found by accident on an imaging test done for another reason. When symptoms appear, they often look like common childhood illnesses: a lasting cough, trouble breathing, chest pain, or repeated lung infections such as pneumonia.
Causes and who gets it
Doctors do not fully understand what causes pleuropulmonary blastoma. Some cases are linked to changes in a gene called DICER1, which normally helps control how cells grow. The condition can run in families, so a child with an affected parent or sibling may face a higher risk. Nothing parents did or did not do causes this tumor.
How doctors diagnose it
Diagnosis usually starts with imaging: a chest X-ray may show a mass, and a CT scan gives a clearer view of the tumor's size and location. To confirm the type, a surgeon often removes part or all of the growth for a pathologist to examine.
Treatment
Treatment depends on the tumor type and how far it has grown. Surgery is the main treatment for most children, and some patients also receive chemotherapy or radiation therapy. Follow-up visits continue for years because some types can come back or change over time.
When to see a doctor
Call a doctor if a child has a cough that lasts for weeks, keeps getting lung infections, breathes fast or with effort, or complains of ongoing chest pain. These signs usually point to something less serious, but they deserve a checkup.
How a direct primary care or concierge doctor fits in
A direct primary care doctor is paid a flat monthly fee instead of billing insurance, while a concierge doctor usually charges more and often works alongside insurance. They can handle follow-up visits, order imaging, and watch for signs the tumor has returned, but they cannot treat the cancer itself and would refer the family to specialists.
Common questions
Is pleuropulmonary blastoma always cancer?
Some early types grow slowly and stay in one place. Other types can spread and need stronger treatment. A pathologist determines the type by studying the removed tissue.
Can adults get it?
It is possible but rare. Almost all cases happen in infants and young children. Adult cases are uncommon enough that doctors often look closely to confirm the diagnosis.
Should family members get genetic testing?
If a child has a DICER1 gene change, parents and siblings may be offered testing and counseling. A genetic counselor can explain what the results mean for the whole family.
Does it come back after treatment?
Some types can return. This is why children need regular follow-up scans and exams for several years. The chance of return depends on the tumor type and how completely it was removed.
Sources
- NIH Genetic and Rare Diseases Information Center - Pleuropulmonary blastoma
- National Cancer Institute - Childhood Cancers - NCI
Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
