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Pleuropulmonary blastoma and lung cysts in babies | CION Cancer Clinics
Pleuropulmonary blastoma is a rare lung tumour of babies and young children that often begins as an air-filled cyst. Most lung cysts in babies are harmless, but a few are early PPB, and most children with PPB carry a DICER1 gene fault. This page explains how the two are told apart, why finding it at the cyst stage matters, and when a genetic test is worth discussing. At CION Cancer Clinics, our oncologists plan screening and care for families with an inherited cancer syndrome, explained in plain words.
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Medically reviewed by Dr. Naresh GunduConsultant Medical Oncologist · MBBS, DNB (Internal Medicine), DM (Medical Oncology, AIIMS) · last reviewed September 2026, next review due September 2027
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On this page
- What is pleuropulmonary blastoma, and why does a lung cyst matter?
- What can a lung cyst in a baby turn out to be?
- How does a cystic PPB change if it is not found?
- The words you will meet, in plain language
- What this page cannot tell you
- Four things parents tell us, and what is actually true
- Common questions about PPB and lung cysts in babies
The short answer
What is pleuropulmonary blastoma, and why does a lung cyst matter?
Pleuropulmonary blastoma, usually shortened to PPB, is a rare lung tumour of babies and young children. It often starts as a thin-walled, air-filled cyst that looks harmless on a scan. Most children with PPB carry a fault in the DICER1 gene, which is why a lung cyst in a baby can lead to a genetic test.
Why the cyst stage matters
In its earliest form, PPB is only a cyst with a thin layer of abnormal cells in its wall. Over months to years, some of these cysts develop solid areas and become more aggressive. Children whose tumour is found and removed at the cyst stage generally do much better than those found later. That single fact drives everything else on this page.
Most lung cysts are not PPB
Lung cysts in babies are usually harmless birth malformations of the airway. Many are picked up on a pregnancy scan and cause no trouble. The difficulty is that a harmless cyst and an early PPB can look almost the same on a scan. A family history, a known DICER1 fault or certain features on the scan help the specialist decide how to handle each one.
A lung cyst in a baby is not a cancer diagnosis. It is a reason to have it assessed by a team that knows PPB.Four possibilities
What can a lung cyst in a baby turn out to be?
Only the specialist, with the scan and often the removed tissue, can tell these apart.
A harmless airway malformation
The most common answer. Part of the lung formed abnormally before birth and left a cyst. Doctors may call it a CPAM. It is not a cancer, though some are removed to prevent infection.
Cystic PPB
Also called type I. The cyst wall contains a thin layer of tumour cells. It is usually found in babies and is the stage at which treatment works best.
A regressed cyst
Some cysts in DICER1 carriers contain no active tumour cells when examined. They are thought to be cysts that stopped growing. They may still be removed or watched.
Solid PPB
Called type II when partly solid and type III when fully solid. These usually appear in toddlers and young children and need more intensive treatment.
May show up as
- Fast or difficult breathing
- A cough or fever that looks like pneumonia
- Chest pain or tiredness in an older child
Not sure whether this applies to you?
Ask an oncologistHow PPB can progress
How does a cystic PPB change if it is not found?
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A cyst forms in infancy
An air-filled cyst appears in one part of the lung, often in the first months of life. The baby may seem entirely well, or have episodes of fast breathing.
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Some cysts stay quiet or shrink back
Not every cyst in a DICER1 carrier goes on to become a solid tumour. Some stop growing. Nobody can yet predict which ones will.
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Solid areas can develop
In others, tumour cells in the wall start to multiply and form solid nodules inside the cyst. This is the partly solid stage, and treatment now usually includes chemotherapy as well as surgery.
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The tumour can become fully solid
Left alone, a solid PPB can fill a large part of the chest. It can spread, most often to the brain or bones. This stage is harder to treat.
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Why early imaging exists
Because the change from cyst to solid tumour happens in early childhood, DICER1 carriers are offered chest scans from infancy. The aim is to find a cyst while it is still only a cyst.
On your report
The words you will meet, in plain language
- Pleuropulmonary blastoma
- A rare childhood tumour of the lung or its lining. Blastoma means it grows from immature, early-type cells.
- CPAM
- Congenital pulmonary airway malformation. A harmless cyst or group of cysts that formed before birth.
- Type I, II and III
- The stages of PPB. Type I is cystic, type II is partly solid, and type III is fully solid.
- Pneumothorax
- Air leaking into the chest outside the lung, making the lung collapse. A burst cyst can cause it.
- Resection
- Surgical removal of the cyst or the part of the lung that holds it, so the tissue can be examined.
- DICER1 carrier
- A person with an inherited DICER1 fault. Most carriers never develop PPB or any serious tumour.
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One thing that cannot wait
A lung cyst can burst and cause the lung to collapse. If a baby or child with a known lung cyst or DICER1 fault suddenly breathes very fast, grunts, struggles to feed, turns pale or blue around the lips, or complains of sudden chest pain, go to the nearest emergency department the same day. Tell the doctors about the cyst. Do not wait to see whether it settles overnight.
Being straight with you
What this page cannot tell you
It cannot tell you what your baby's cyst is. That needs a scan read by a radiologist who sees children's lungs often, and very often the removed tissue examined by a pathologist who knows PPB. Some pathology slides are sent to an international expert registry for a second opinion, which is normal and worth asking about.
It cannot read a DICER1 report
What your specific variant means is a question for the counsellor who ordered the test. A normal blood result does not always rule out PPB in a cyst that has already been removed, and your team will explain why.
Who this does not apply to
Most babies with a lung cyst seen on a pregnancy scan have a harmless malformation and no DICER1 fault. Most children with pneumonia do not have a hidden tumour. Genetic testing is considered when a cyst has unusual features, when PPB is confirmed, or when a relative carries the fault.
The evidence is limited
PPB is rare everywhere, and most of what is known comes from a registry of a few hundred children, largely outside India. Decisions on whether to remove every cyst are still debated. Your child's surgeon and oncologist should explain their reasoning, and a counsellor can go over it again in Telugu.
Commonly believed
Four things parents tell us, and what is actually true
"The cyst was seen before birth, so it must be harmless."
Most such cysts are harmless, but cystic PPB can also be present before birth. The cyst still needs proper follow-up after delivery.
"Our baby is breathing normally, so the cyst can wait."
Early PPB rarely causes symptoms. The absence of symptoms is not reassurance on its own. Follow the plan your specialist sets.
"Once the cyst is removed, the gene problem is over."
Removing the cyst treats that cyst. If your child carries a DICER1 fault, new cysts and other linked growths are still possible, so a check plan continues.
"PPB happened because of something we did in pregnancy."
Nothing you ate, did or avoided caused this. It comes from a gene fault and chance events inside one cell. Neither parent is to blame.
Questions we are asked
Common questions about PPB and lung cysts in babies
How is a lung cyst found in a baby?
Sometimes on a pregnancy ultrasound, sometimes on a chest X-ray taken for breathing trouble or a chest infection, and sometimes on a scan arranged because a relative carries DICER1. A CT scan of the chest then shows the cyst in detail.
Does every lung cyst need an operation?
Not always. Many specialists recommend removing cysts in DICER1 carriers because an early PPB cannot be ruled out by scan alone. For other cysts the decision depends on size, symptoms and features on imaging. Ask your surgeon to explain the choice.
Is surgery on a baby's lung safe?
Lung surgery in infants is done regularly by paediatric surgical teams, and most babies recover well. Your surgeon will explain the specific risks for your child. The lungs continue to grow after surgery, which helps make up for the removed part.
What treatment does PPB need?
A cystic PPB is usually treated with surgery, sometimes followed by chemotherapy. Partly solid and solid types need surgery and chemotherapy together. Treatment is planned by a paediatric oncologist who follows international guidance for this tumour.
Should my baby be tested for DICER1?
If PPB is confirmed, yes, genetic testing is recommended. If a cyst looks like a typical harmless malformation and there is no family history, testing may not be needed. A genetic counsellor can help you weigh this.
If our child has DICER1, what about our next baby?
First, both parents are tested for the child's fault. If one carries it, each future child has a one in two chance of inheriting it. Discuss options with a counsellor before the next pregnancy if you can.
Can PPB come back after treatment?
It can, particularly after the solid types, which is why follow-up scans continue after treatment. A child with DICER1 also needs checks of the kidneys, thyroid and other organs as they grow.
Where can we get this assessed near Hyderabad?
A paediatric surgeon and a paediatric oncologist should see any baby with a suspicious lung cyst. If you already have scan reports or a DICER1 result, a genetic counsellor can help coordinate next steps. Call the CION helpline to be pointed to the right team.
Your Specialists
Meet CION's oncologists. Bring your family history or genetic report to them.
Our medical oncologists see people with a strong family history of cancer, arrange genetic counselling and testing where it fits, and plan the checks that follow.
Medical Oncologist
Dr. C. Raghavendra Reddy
MBBS(Gold Medal), DNB(General Medicine), DM(Medical Oncology)(Gold Medal)
Medical Oncologist
Dr. Bharati Devi Gorantla
MBBS, MD(General Medicine), DM(Medical Oncology)(Adyar,Chennai), ECMO, MRCP SCE(UK)
Medical Oncologist
Dr. Owais Mohammed
MBBS, MD (General Medicine), DrNB (Medical Oncology), ECMO, MRCP SCE (Medical Oncology) (UK)
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Sources
- GeneReviews (NCBI) — DICER1-Related Tumor Predisposition
- MedlinePlus Genetics — DICER1 syndrome
- National Cancer Institute — Unusual Cancers of Childhood Treatment (PDQ) - Patient Version
- MedlinePlus Genetics — DICER1 gene
This page is general information, not a prescription. Do not change or stop any treatment based on what you read here. If anything is worrying you, contact your own treating team — or call our helpline and we will help you reach the right specialist.
Keep reading
Related pages
DICER1 syndrome explainedThe gene behind most cases of PPBDICER1 surveillance in children and adultsThe chest scans and other checks carriers are offeredDICER1 syndrome in children: what to watch forOther childhood signs worth knowingTesting the family for DICER1Whether parents and siblings should be tested
Talk to us
Has a lung cyst been found in your baby?
We can review the scans and reports with you and help you reach the right paediatric and genetics teams. Counselling is available in Telugu. One helpline serves every CION centre.