Health Encyclopedia

 

 

Pleuropulmonary Blastoma

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Pleuropulmonary Blastoma is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • PPB
  • pneumoblastoma
  • mesenchymal cystic hamartoma
  • cystic mesenchymal hamartoma
  • pulmonary rhabdomyosarcoma
  • rhabdomyosarcoma in lung cyst
  • pediatric pulmonary blastoma

Disorder Subdivisions

  • PPB family tumor susceptibility syndrome

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". PPB occurs almost exclusively in children under the age of approximately 7-8 years and rarely in older children, in teenagers and more rarely in adults. Three subtypes of PPB exist and are called type I, type II, and type III PPB. Type I PPB takes the form of cysts in the lungs (air-filled pockets) and occurs in the youngest children with PPB (from birth to about 2 years of age). Type II PPB has cysts and solid tumor. Type III PPB is entirely solid tumor. Types II and III PPB tend to occur after age 2 years. Children with type I PPB can have the disease come back. If type I PPB comes back, it usually happens within about 4 years of the original diagnosis, and it is usually type II or III when it recurs. Children with type I PPB have a better outlook ("prognosis") than children with types II and III PPB; most type I PPB patients are cured (more than 80%). Treatment for type I consists of surgery and possibly chemotherapy; for Types II and III PPB surgery, chemotherapy and possibly radiation therapy is recommended. At present, about 50-60% of types II and III children 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. PPB is related to, or in the broad family of, cancers such as cancers of muscle, cartilage, and connective tissues. Physicians classify diseases this way in order to compare features and to compare treatments. Often treatments that are effective for one member of a family of cancers are effective for other members of that family.



PPB occurs in the lungs so PPB can be called a lung cancer, but PPB has no connection whatsoever with lung cancers in adults that are often related to tobacco use.



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. Less common metastasis occurs in the bones, the liver, remaining parts of the lung, and other organs. It is not known why PPB has this pattern of spread to brain, liver, or bones. PPB can also spread by growing directly into tissues next to the lung like the diaphragm.



PPB family cancer syndrome: In about 25% of children with PPB, there are other childhood cancers or abnormalities in the PPB patient or in their families. This is called the PPB family cancer syndrome. This can include lung cysts, kidney cysts, ovarian or testicular tumors in children or young adults, thyroid tumors (sometimes malignant), and various other cancers and non-malignant (non cancerous) diseases. It is not known what causes the abnormalities in these children and families, but research is underway to discover the presumed genetic basis for this tendency to childhood cancers or other abnormalities. It should be emphasized that many children and adults in these families have no medical problems.

Resources

Rare Cancer Alliance

1649 North Pacana Way

Green Valley, AZ 85614

USA

Internet: http://www.rare-cancer.org



International Pleuropulmonary Blastoma Registry

Children's Hospitals and Clinics of Minnesota

2545 Chicago Ave. S.

Suite 412

Minneapolis, MN 55404

USA

Tel: (612)813-7115

Fax: (612)813-7108

Email: gretchen.williams@childrensmn.org

Internet: http://www.ppbregistry.org



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

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Cancer.Net

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

Email: contactus@cancer.net

Internet: http://www.cancer.net/



BeatSarcoma

76 Ellsworth Street

San Francisco, CA 94110

Tel: (415)826-0474

Email: info@beatsarcoma.org

Internet: http://www.beatsarcoma.org/



Pleuropulmonary Blastoma Research at Children's National Medical Center

111 Michigan Ave. NW

Washington, DC 20010

Tel: (202)476-2815

Email: dashill@cnmc.edu

Internet: http://www.ppbgeneticstudy.org/collaborators/collaborators.html or http://www.ppbgeneticstudy.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org 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. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

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.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  2/7/2013

Copyright  2005, 2010, 2013 National Organization for Rare Disorders, Inc.

This information does not replace the advice of a doctor. Healthwise, Incorporated disclaims any warranty or liability for your use of this information. Your use of this information means that you agree to the Terms of Use. How this information was developed to help you make better health decisions.

Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Healthwise, Incorporated.