What Is Pleuropulmonary Blastoma (PPB)?
Pleuropulmonary blastoma (PPB) is a rare but aggressive type of cancer that occurs in early childhood. It develops in the lungs or the pleura—the thin layer of tissue lining the lungs. PPB most often affects children under the age of six. Tumours can present in different forms: they may be fluid-filled (cystic), solid, or a combination of both.
Causes
The exact cause of PPB is not always known, but many cases have been linked to mutations in the DICER1 gene. This genetic change can be inherited, meaning it may run in families and increase the risk of developing other types of tumours.
Who Is Affected and How Rare Is It?
PPB is extremely rare and accounts for only a small portion of childhood lung cancers. Nearly all cases are diagnosed in children younger than six years old.
Signs and Symptoms
Symptoms of PPB can resemble those of common lung infections, making diagnosis challenging. Children may experience:
- Difficulty breathing
- Persistent cough or wheezing
- Chest pain
- Unexplained fever or symptoms similar to pneumonia
Diagnosis
To diagnose PPB, doctors typically begin with imaging tests to examine the lungs:
- Chest X-rays or CT scans help determine the size, location, and composition (cystic, solid, or mixed) of the tumour.
- A biopsy—removal of a tissue sample for microscopic analysis—is necessary to confirm the diagnosis.
Types of PPB
PPB is categorised into three types, depending on the tumour’s appearance:
- Type I: Mostly cystic; considered the least aggressive form.
- Type II: Contains both cystic and solid areas; more aggressive.
- Type III: Entirely solid; the most aggressive and difficult to treat.
Treatment
Treatment depends on the tumour type and stage:
- Surgery is typically the first line of treatment for Type I tumours and aims to remove the tumour completely.
- Chemotherapy is often used in more aggressive cases (Types II and III), either before surgery to shrink the tumour or after surgery to reduce the risk of recurrence.
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