Pleuropulmonary Blastoma



What is a Pleuropulmonary Blastoma?

Pleuropulmonary blastoma (PPB) is a very rare cancer of the chest, which is usually observed in children under 5 years of age. It originates from lung and pleura. The cancer mainly affects one lung but can spread to the pleura (the linings of the chest), the mediastinum (soft tissues in the center of the chest) or the diaphragm.

Who gets Pleuropulmonary Blastoma and why? 

Usually PPB affects very young children. The cause is unknown but some children have a genetic disease that affects a gene called DICER1 which predisposes to PPB and other rare cancers. Lung malformations, such as a congenital cystic adenomatoid disease (CCAM) may be associated with PPB.

Is there a risk for brothers / sisters to develop the same cancer or other types of cancer? 

In families with specific genetic diseases described above, the sisters and brothers can harbor the same genetic anomaly and be predisposed to PPB and to other rare tumors. Genetic investigations and counseling are recommended in this disease. In this situation, some other locations, such as the thyroid or ovary, should be checked on a regular basis.

Are Pleuropulmonary Blastomas all the same?

PPB is classified into three different subtypes:

  • Type 1 – the tumor has only cystic areas and has better prognosis;
  • Type 2 – the tumor has both cystic and solid areas;
  • Type 3 – the tumor has only solid areas and is more difficult to treat.

What are the typical symptoms and signs of Pleuropulmonary Blastoma? 

PPB may present with difficulty in breathing, fever, cough, chest pain or weakness. In most cases the initial diagnosis is confused with persistent bronchitis or pneumonia.

Which investigations are necessary for a child with Pleuropulmonary Blastoma?

The first examination which raises the suspicion of PPB is usually the chest x-ray or, sometimes, chest ultrasound. Afterwards, your child may need further tests to check the size and location of the cancer and whether it has spread to other parts of the body. These tests may include:

  • MRI (magnetic resonance imaging) – this method uses magnetism to build up a very detailed picture of the body.
  • CT (computerized tomography) scan – this method uses x-rays to build up a three-dimensional picture the body.

Are there different stages of the disease? 

PPB can be localized (involving only the lung and the pleura) or disseminated, if the cancer cells spread to other parts of the body (metastatic disease).

What about the Pleuropulmonary Blastoma treatment?


In some cases, especially in type I PPB, the initial surgery with complete removal of the tumor is possible. When the tumor in the chest is too big initially to be removed (type II or III) or when there is a distant spread of disease, a biopsy of the tumor is usually performed at the beginning. Surgery may occur later, after chemotherapy has been given to shrink the tumor size.


PPB are sensitive to chemotherapy drugs. The role and the type of chemotherapy is not yet completely defined, but it seems to improve the chances of long-term cure. Most children with type II or III PPB receive chemotherapy, especially when surgical removal of the tumor at diagnosis is not feasible.


The role of radiotherapy remains controversial and the experts in multidisciplinary meetings will usually discuss if it is useful for your child.

What are the results of treatment?

The most important factors that determine a good outcome is the absence of distant spread and a complete surgical removal of the tumor. The best outcome is with type I PPB.

What research is carried out for Pleuropulmonary Blastoma?

PPB is a very rare tumor, which makes the research difficult. Nevertheless, there are groups of experts in the USA and Europe, which promote clinical and scientific research on PPB. Specific genetic alterations have been found that allow to identify patients and families who are at risk of developing PPB and other rare tumors.

What EXPeRT is doing for children with Pleuropulmonary Blastoma?

The EXPeRT group is working for children with PPB in many ways:

  • Collecting data of children with PPB from all European countries
  • Creating guidelines for diagnosis and treatment of PPB with the goal to optimize the chance of cure for all children
  • Providing advice in difficult cases of PPB to the leading clinician, both at a European level and worldwide
  • Collaborating with other Groups of experts and researchers to increase the knowledge on PPB.