What Is Pleuropulmonary Blastoma?

A pleuropulmonary blastoma is a rare form of lung cancer that most frequently occurs in children. This kind of pediatric cancer develops inside the lungs or from the membranes that cover them. It may also arise from structures next to the lungs, such as the heart, major arteries or diaphragm. Pleuropulmonary blastoma is a serious condition, and only a minority of cases are curable. Treatment usually consists of surgery, which can be used to remove the section of lung containing the tumor, and this is often combined with chemotherapy.

Doctors are still researching pleuropulmonary blastoma causes, and it is not fully understood why this type of cancer develops. It can affect children of a variety of ages, including newborns, and is also occasionally found in adults. There is an association between this kind of cancer and cysts found in the lungs. Babies with lung cysts are more likely to develop pleuropulmonary blastoma in the future. The disease also occurs more frequently in those who have relatives with cancer, particularly siblings who already have the same blastoma of the lungs.

Pleuropulmonary blastoma symptoms are variable, and may resemble those associated with other diseases. Some children may cough, become feverish or lose weight. There may be an obvious lump in the chest, or shortness of breath could occur. Some children may have more respiratory infections than usual, which could keep recurring.

If symptoms suggest pleuropulmonary blastoma, doctors may carry out tests such as chest X-rays and CT scans to make a diagnosis. A flexible tube with a camera may be used to investigate the lungs from inside. The diagnostic process involves classifying a pleuropulmonary blastoma into one of three possible types.

The first, and least severe, type of tumor, known as Type I, tends to appear in the form of a hollow cyst, while the second, more serious type II has a mixture of hollow and solid areas. At the most serious end of the scale, a tumor described as Type III is an entirely solid mass. This is the type most likely to spread to other parts of the body, while Type I is least likely to do so. Type II lies midway between the other two in its outlook and tendency to spread.

Pleuropulmonary blastoma treatment usually consists of surgery to remove the tumor and the section of lung that contains it. Chemotherapy is normally given as well to kill off any remaining cancer cells. This treatment can cure some Type I tumors. The outlook is less positive for Type II and Type III tumors, which are more likely to spread quickly through the body and become incurable.