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Pleuropulmonary Blastoma Treatment

Pleuropulmonary blastoma (PPB) is a rare form of lung cancer. It typically affects children under age 6. 

The cause is not always known. But certain genetic changes may increase the risk of PPB. Some children with PPB have a change in the DICER1 gene or a family history of DICER1 syndrome. 

The severity of PPB depends on its type. There are 4 types:

  • Type 1: Contains air-filled pockets (cysts) with early signs of cancer. It is most often seen in children under age 3. 
  • Type 1r: Similar to Type 1 but does not have cancer cells. The “r” stands for “regressed.” This means the cysts may become smaller or not grow into cancer.
  • Type 2: Includes both cysts and solid cancer parts
  • Type 3: Aggressive, solid tumors 

Knowing the type is important. It helps doctors choose the best treatment plan.

Treatment of pleuropulmonary blastoma

Treatment for PPB depends on the tumor type. It may include:

  • Surgery: Removing the tumor is often the first step. For children with Type 1 or Type 1r PPB, surgery may be all that is needed. The care team closely follows the patient’s cancer to see if the tumor comes back. 
  • Chemotherapy: For children with Type 2 or Type 3 PPB, chemotherapy is used to shrink the tumor before surgery. Medicines may include cyclophosphamide, doxorubicin, ifosfamide, topotecan, and vincristine.
  • Radiation therapy: Some children with Type 3 PPB may also need radiation to target cancer cells.

Early diagnosis and treatment can improve outcomes for children with this disease.

Pleuropulmonary blastoma clinical trials

St. Jude offers clinical trials and cancer research studies for children, teens, and young adults with pleuropulmonary blastoma. Learn more about clinical research at St. Jude.

Recruiting
ARAR2331: Pleuropulmonary Blastoma Treatment

Study goal:

To standardize treatments for all types of pleuropulmonary blastoma and find safer, more effective ways to treat children with this rare lung cancer

Age:

Under age 6

Pleuropulmonary blastoma care at St. Jude

St. Jude provides the highest quality of care for patients with pleuropulmonary blastoma:

  • St. Jude offers a dedicated team of specialists to meet the needs of children with pleuropulmonary blastoma, including:
  • Complete, quality surgery is an important part of treating pleuropulmonary blastoma. Cancer surgery in children requires expertise because of the risks involved. Excellence in care before, during, and after surgery is key. The expert skills and experience of St. Jude specialty surgeons help improve patients’ chances for best outcomes. 

More reasons to choose St. Jude for care include:

  • We are consistently ranked among the best childhood cancer centers in the nation by US News & World Report. 
  • At St. Jude, we have created an environment where children can be children and families can be together.  
  • We lead more clinical trials for childhood cancer than any other hospital in the U.S.  
  • St. Jude is the only National Cancer Institute–designated Comprehensive Cancer Center just for children. A Comprehensive Cancer Center meets rigorous standards for research that develops new and better approaches to prevent, diagnose, and treat cancer. 
  • The nurse-to-patient ratio at St. Jude is about 1:3 in hematology and oncology and 1:1 in the Intensive Care Unit. 
  • Patients may be able to get expert, compassionate care and treatment closer to their homes through the St. Jude Affiliate Program. 
A statue of children running and holding hands

Seeking treatment at St. Jude

Patients accepted to St. Jude must have a disease we treat and must be referred by a physician or other qualified medical professional. We accept most patients based on their ability to enroll in an open clinical trial.

How to seek treatment

Contact the Physician / Patient Referral Office

Submit online referral form

Call: 1-888-226-4343 (toll-free) or 901-595-4055 (local)  | Fax: 901-595-4011 | Email: referralinfo@stjude.org | 24-hour pager: 1-800-349-4334

The solid tumor coordinator is dedicated to helping you seek treatment or refer a patient.

Solid Tumor Coordinator

 

Learn more

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