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Pleuropulmonary Blastoma

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Posted by | Feb 25, 2025

What is Pleuropulmonary Blastoma (PPB)?

Pleuropulmonary Blastoma (PPB) is a rare lung cancer that develops in children, primarily affecting infants and young kids. It starts in the developing lung tissue and can grow at different rates. Doctors classify PPB into three types—Type I, II, and III—based on how advanced and aggressive the tumour is. Early detection and treatment are essential for improving outcomes.

Causes

PPB is strongly linked to mutations in the DICER1 gene, which is involved in cell growth and development. In some cases, a family history of PPB or other DICER1-related tumours increases the risk. However, no specific environmental factors have been identified as causes of this cancer.

Signs and Symptoms

Children with PPB may experience:

  • Respiratory distress (shortness of breath, wheezing)
  • Chest pain
  • Persistent cough
  • Pleural effusion (fluid buildup around the lungs)

Diagnosis

Diagnosing PPB involves multiple tests:

  • Imaging: Chest X-rays and CT scans help detect abnormal lung masses.
  • Biopsy & Molecular Testing: A tissue sample is examined to confirm the presence of the DICER1 gene mutation, commonly associated with PPB.
  • Genetic Screening: If there is a family history of DICER1-related conditions, genetic testing is recommended to assess potential risks early.

Treatment

Treatment depends on the stage and type of PPB:

  • Surgery is the primary treatment for localised tumours, aiming to remove the cancer completely.
  • Chemotherapy (using drugs like Vincristine, Actinomycin D, and Cyclophosphamide) is often used to shrink tumours before surgery or treat more advanced cases.
  • Radiation Therapy is reserved for severe or recurrent cases when surgery and chemotherapy are not fully effective.

Prognosis

The outlook for PPB varies depending on the tumour type:

  • Type I has a good prognosis and is often successfully treated with surgery alone.
  • Type II and III are more aggressive and harder to treat, leading to a poorer prognosis despite intensive therapy.

However, early diagnosis and timely treatment significantly improve survival rates, making early detection crucial.

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