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. What Causes PPB? The cause of PPB is not known. Most often, it develops randomly. About 40% of the children with PPB have had or have a family history of lung cysts or kidney cysts, rare ovarian tumors (Sertoli-Leydig cell tumors), leukemia, or polyps. Gene mutations are rarely found. (Mutations in the Dicer1 gene have been reported in familial cases of PPB.) What Is the Common Treatment and Outcome for PPB? The treatment for children with PPB will depend on the stage, where it has spread, and whether it is affecting organ function. Surgery is the main treatment for PPB, and the goal is to completely remove the tumor. If it cannot be completely removed, chemotherapy and sometimes radiation therapy are used to treat PPB. In the following sections, tests and treatments that may be used to diagnose and treat rare cancers will be discussed to help you learn about your child’s care. Your treatment team will discuss the specific tests and treatments for cancer.
n WHAT IS METASTASIS? Metastasis refers to the spread of a tumor from its primary site (original location) to other parts of the body. If a biopsy reveals cancer, more tests will be performed to see if the cancer has spread to other organs.
n WHAT IS STAGING? Understanding the staging systems used to describe rare tumors is important for you and your child. Staging is the process of determining the location and amount of the disease at the time of diagnosis to make recommendations for treatment. The higher the stage number, the more the disease has spread. There is no one staging system that applies to all rare tumors. Some staging systems are developed just for a particular type of cancer. The common elements considered in most staging systems are • site of the primary tumor • tumor size and number of tumors • lymph node involvement (spread of cancer into lymph nodes) • cell type and tumor grade (how closely the cancer cells resemble normal tissue cells) • the presence or absence of metastasis.
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