Overview Pleuropulmonary blastomas are rare tumors that usually occur under the tissue covering the lungs.
Changes in a specific chromosome may be found in pleuropulmonary blastoma. Every cell in the body contains DNA (genetic material stored inside chromosomes) that determines how the cell looks and acts. Part of the DNA in chromosome 8 is often abnormal in these tumors.
Early-stage pleuropulmonary blastoma develops as a cyst-like tumor in the lung. It usually appears in the first 2 years of life and has a good prognosis. Late-stage tumors are solid and can spread to the brain. When the tumor can be completely removed by surgery at any time during treatment, the chance of recovery is improved. The tumor may recur or spread, in spite of surgical removal.
There are no standard treatment options for pleuropulmonary blastoma. Early-stage tumors may be treated with surgery alone, or surgery with adjuvant chemotherapy to help prevent the tumor from coming back. Late-stage tumors may be treated with surgery followed by chemotherapy. Radiation may be used when the tumor cannot be surgically removed. A family history of cancer in close relatives has been noted for many young patients affected by this tumor.
Pleuropulmonary blastoma may also occur in siblings of patients.
