Pleuropulmonary Blastoma (PPB)

Definition

Pleuropulmonary blastoma (PPB) is a rare and aggressive type of childhood lung cancer that begins in the lung tissue or the pleural cavity. It typically affects infants and young children under the age of six.

Characteristics

PPB is classified into three types:

• Type I: purely cystic and generally less aggressive
• Type II: mixed cystic and solid
• Type III: entirely solid and more aggressive
  Symptoms may include coughing, difficulty breathing, chest pain, fever, or a noticeable mass in the chest.

Diagnosis and Prognosis

Diagnosis involves imaging tests such as chest X-ray, CT scan, and MRI, followed by biopsy. Genetic testing for DICER1 mutations is often recommended. Prognosis depends on the type and stage of the tumor, with Type I having a better outcome than Type II or III.

Treatment

Treatment typically includes surgical removal of the tumor and chemotherapy. In more advanced or aggressive cases, radiation therapy may also be used. Lifelong monitoring is often required due to the risk of recurrence or other DICER1-related tumors.

Last Revised: 12 May , 2025