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Pleuropulmonary Blastoma

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.

WebMD Medical Reference from the National Organization of Rare Disorders

Last Updated: February 25, 2014
This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.

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