Rare Tumors Patient & Family Handbook

the tumor is usually the main treatment. Chemotherapy or radiation therapy may be used if the cancer has spread to other areas. Hormone regulation and replacement therapy will be an important part of treatment, along with follow-up care with an endocrinologist.

PLEUROPULMONARY BLASTOMA

WHAT IS PLEUROPULMONARY BLASTOMA? Pleuropulmonary blastoma (PPB) is a rare type of childhood lung cancer. PPB begins in the tissues of the pleura (a thin layer of tissue that covers the lungs and lines the interior wall of the chest cavity) or in the lung tissue itself (pulmonary). There are four main types of PPB: Type I is made up of cysts (air pockets) with evidence that the cysts are cancerous. This usually occurs in very young children, usually around 10 months old. Type Ir (the “r” stands for regressing) is similar to type I but does not have cancerous cells. Types II and III generally occur in children ages 3–4 years. They are cancerous and require intensive chemotherapy.

WHAT ARE SOME OF THE SIGNS AND SYMPTOMS OF PPB?

PPB is so rare that doctors often think the symptoms are caused by a common childhood illness and may overlook the diagnosis of PPB. The two common sets of symptoms that may indicate PPB are sudden, strained breathing cough, fever, chest pain, or symptoms of pneumonia.