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Pleuropulmonary Blastoma - Child

To view this article in the new Rare Cancers Australia Knowledgebase, click here 

Pleuropulmonary Blastoma

Pleuropulmonary blastomas (PPBs) form in the tissue of the lung and pleura (tissue that covers the lungs and lines the inside of the chest). PPBs can also form in the organs between the lungs including the heart, aorta, and pulmonary artery, or in the diaphragm (the main breathing muscle below the lungs).

There are three stages of PPB that are described as types:

  • Type I tumors are cyst -like tumors in the lung. They are most common in children aged 2 years and younger and can usually be cured.
  • Type II tumors are cyst-like with some solid parts. These tumors sometimes spread to the brain.
  • Type III tumors are solid. These tumors often spread to the brain.

Risk Factors, Symptoms, and Diagnostic and Staging Tests

The risk of PPB is increased by the following:

  • Having a family history of any type of cancer in close relatives.
  • Having a brother or sister with PPB.
  • Having a personal history of other types of cancer.

PPB may cause any of the following signs and symptoms. Check with your child’s doctor if you see any of the following problems in your child:

  • A cough that doesn’t go away.
  • Trouble breathing.
  • Chest discomfort.
  • Wheezing.
  • Streaks of blood in sputum (mucus coughed up from the lungs).
  • Hoarseness.
  • Pain under the rib cage.
  • Pain, swelling, or lumps in the abdomen.
  • Loss of appetite.
  • Weight loss for no known reason.
  • Feeling very tired.

Other conditions that are not PPB may cause these same symptoms.

Tests to diagnose and stage PPB may include the following:

  • Physical exam and history.
  • X-ray of the chest.
  • CT scan.
  • PET scan.

Other tests used to diagnose PPB include the following:

  • Bronchoscopy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
  • Thoracoscopy: A surgical procedure to look at the organs inside the chest to check for abnormal areas. An incision (cut) is made between two ribs, and a thoracoscope is inserted into the chest. A thoracoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of cancer. In some cases, this procedure is used to remove part of the esophagus or lung. If the thoracoscope cannot reach certain tissues, organs, or lymph nodes, a thoracotomy may be done. In this procedure, a larger incision is made between the ribs and the chest is opened.

PPBs may spread or recur (come back) even after being removed by surgery.

Treatment

Treatment of pleuropulmonary blastomas in children is usually surgery to remove the whole lobe of the lung the tumor is in, with or without chemotherapy.

For more information on Pleuropulmonary Blastoma click here

This link is to the National Cancer Institute (NCI) cancer website in the United States. There may be references to drugs and clinical trials that are not available here in Australia.

For information about clinical trials that are available in Australia click here

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