Pleuropulmonary Blastoma

National Organization for Rare Disorders, Inc.

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It is possible that the main title of the report Pleuropulmonary Blastoma is not the name you expected.

Disorder Subdivisions

  • None

General Discussion

Pleuropulmonary blastoma (PPB) is a rare childhood cancer occurring in the chest, specifically in the lungs or in the coverings of the lungs called "pleura". Three subtypes of PPB exist and are called Type I, Type II, and Type III PPB. Type I PPB takes the form of one or more cysts in the lungs (air-filled pockets) and may be found in very young children with PPB (from birth to about 2 years of age). Type III PPB is entirely solid tumor. Type II PPB includes both cystic and solid parts. Types II and III PPB tend to be found more often after 2 years of age. Type Ir (the "r" stands for regressed/ regressing) is another type of PPB. Under the microscope Type Ir is similar to Type I PPB, but it does not have cancerous cells. Type I, II, and III PPB are usually found in children under the age of approximately 7-8 years; PPB occurs rarely in older children or teenagers, and even more rarely in adults, but Type Ir PPB may be found at any age. Children with Type I have a better outlook ("prognosis") than children with Types II and III PPB; most Type I PPB patients are cured (89%) but Type I PPB can sometimes recur ("come back") as Type II or III PPB. Treatment for Type I consists of surgery and possibly chemotherapy. Treatment for Types II and III PPB consists of surgery and chemotherapy and possibly radiation therapy. At present, about 50-70% of children with Types II and III PPB are cured.

PPB is a childhood cancer in the family of cancers called soft tissue cancers, which are scientifically called sarcomas. PPB is, therefore, a soft tissue sarcoma. Physicians classify diseases this way in order to compare features and to compare treatments.

PPB occurs in the lungs and is the most common lung cancer of childhood but PPB has no connection to lung cancers in adults that are often related to tobacco use or asbestos exposure.

Like many cancers, PPB can spread through the blood to other areas of the body. When a cancer spreads to another part of the body it is called a "metastasis" of the cancer. Types II and III PPB can metastasize. The most common location for a PPB metastasis is the brain. PPB may also spread to remaining part of the lung, bones, liver and rarely to other organs. PPB can also spread by growing directly into tissues next to the lung like the diaphragm.

Supporting Organizations


76 Ellsworth Street
San Francisco, CA 94110
Tel: (415)826-0474


American Society of Clinical Oncology
2318 Mill Road Suite 800
Alexandria, VA 22314
Tel: (571)483-1780
Fax: (571)366-9537
Tel: (888)651-3038

Focus On Rhabdo

100 Middlesex Blvd. 08536
Ste 212
Plainsboro, NJ 8536
Tel: (818)554-6480
Fax: (609)799-4321

Genetic and Rare Diseases (GARD) Information Center

PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311

International Pleuropulmonary Blastoma Registry

Children's Hospitals and Clinics of Minnesota
2545 Chicago Ave. S.
Minneapolis, MN 55404
Tel: (612)813-7115
Fax: (612)813-7108

Jared's Juggernaut, Inc.


Pleuropulmonary Blastoma Research at Children's National Medical Center

111 Michigan Ave. NW
Washington, DC 20010
Tel: (202)476-2815

Rare Cancer Alliance

1649 North Pacana Way
Green Valley, AZ 85614

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to and click on Rare Disease Database under "Rare Disease Information".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only.

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This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

Last Updated:  4/25/2016
Copyright  2016 National Organization for Rare Disorders, Inc.