Pleuropulmonary blastoma
Encyclopedia
Pleuropulmonary blastoma (PPB) is a rare cancer
originating in the lung
or pleural cavity
. It occurs most often in infants and young children but also has been reported in adults. In a retrospective review of 204 children with lung tumors, pleuropulmonary blastoma and carcinoid tumor were the most common primary tumors (83% of the 204 children had secondary tumors spread
from cancers elsewhere in the body). Pleuropulmonary blastoma is regarded as malignant.
An international registry has been established.
To find out more information PPB Registry has the biggest information section on Pleuropulmonary Blastoma in the world.
ing, an upper respiratory tract infection
, shortness of breath, and chest pain
. These symptoms are very non-specific, and can be caused by other types of tumor in the lung or mediastinum
more generally, and by other conditions. Imaging (X-ray, CT, MRI) may be used to determine the presence and precise location of a tumor, but not a specific diagnosis of PPB or other tumor.
Doctors are unable to tell if a child has PPB right away, and not upper respiratory tract infection
, until more test are taken and they show that there is no infection. Another symptom is pneumothorax
.
Type I PPB is made up of mostly cysts, and may be hard to distinguish from benign lung cysts, and there is some evidence that not all type I PPB will progress to types II and III. Types II and III are aggressive, and cerebral metastasis is more frequent in PPB than in other childhood sarcoma
s.
. Other tests like x-rays, CAT scans, and MRI's can suggest that cancer is present, but only an examination of a piece of the tumor can make a definite diagnosis.
, whether the cancer
has spread, and the child's overall health. Surgery is the main treatment for PPB. The main goal of surgery is to remove the tumor
. If the tumor is too large to be completely removed, or if it's not possible to completely remove the tumor, surgery may be performed after chemotherapy
. Because PPB can return after treatment, regular screening for possible recurrence should continue for 48 to 60 months, after diagnosis.
Cancer
Cancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...
originating in the lung
Lung
The lung is the essential respiration organ in many air-breathing animals, including most tetrapods, a few fish and a few snails. In mammals and the more complex life forms, the two lungs are located near the backbone on either side of the heart...
or pleural cavity
Pleural cavity
In human anatomy, the pleural cavity is the potential space between the two pleura of the lungs. The pleura is a serous membrane which folds back onto itself to form a two-layered, membrane structure. The thin space between the two pleural layers is known as the pleural cavity; it normally...
. It occurs most often in infants and young children but also has been reported in adults. In a retrospective review of 204 children with lung tumors, pleuropulmonary blastoma and carcinoid tumor were the most common primary tumors (83% of the 204 children had secondary tumors spread
Metastasis
Metastasis, or metastatic disease , is the spread of a disease from one organ or part to another non-adjacent organ or part. It was previously thought that only malignant tumor cells and infections have the capacity to metastasize; however, this is being reconsidered due to new research...
from cancers elsewhere in the body). Pleuropulmonary blastoma is regarded as malignant.
History
Pleuropulmonary blastoma was first described in 1988.An international registry has been established.
To find out more information PPB Registry has the biggest information section on Pleuropulmonary Blastoma in the world.
Signs and symptoms
Symptoms may include coughCough
A cough is a sudden and often repetitively occurring reflex which helps to clear the large breathing passages from secretions, irritants, foreign particles and microbes...
ing, an upper respiratory tract infection
Upper respiratory tract infection
Upper respiratory tract infections are the illnesses caused by an acute infection which involves the upper respiratory tract: nose, sinuses, pharynx or larynx...
, shortness of breath, and chest pain
Chest pain
Chest pain may be a symptom of a number of serious conditions and is generally considered a medical emergency. Even though it may be determined that the pain is non-cardiac in origin, this is often a diagnosis of exclusion made after ruling out more serious causes of the pain.-Differential...
. These symptoms are very non-specific, and can be caused by other types of tumor in the lung or mediastinum
Mediastinum
The mediastinum is a non-delineated group of structures in the thorax, surrounded by loose connective tissue. It is the central compartment of the thoracic cavity...
more generally, and by other conditions. Imaging (X-ray, CT, MRI) may be used to determine the presence and precise location of a tumor, but not a specific diagnosis of PPB or other tumor.
Doctors are unable to tell if a child has PPB right away, and not upper respiratory tract infection
Upper respiratory tract infection
Upper respiratory tract infections are the illnesses caused by an acute infection which involves the upper respiratory tract: nose, sinuses, pharynx or larynx...
, until more test are taken and they show that there is no infection. Another symptom is pneumothorax
Pneumothorax
Pneumothorax is a collection of air or gas in the pleural cavity of the chest between the lung and the chest wall. It may occur spontaneously in people without chronic lung conditions as well as in those with lung disease , and many pneumothoraces occur after physical trauma to the chest, blast...
.
Types
Pleuropulmonary blastoma is classified into 3 types:- Type I is multicystic
- Type II shows thickening areas (nodules) within this cystic lesion
- Type III shows solid masses.
Type I PPB is made up of mostly cysts, and may be hard to distinguish from benign lung cysts, and there is some evidence that not all type I PPB will progress to types II and III. Types II and III are aggressive, and cerebral metastasis is more frequent in PPB than in other childhood sarcoma
Sarcoma
A sarcoma is a cancer that arises from transformed cells in one of a number of tissues that develop from embryonic mesoderm. Thus, sarcomas include tumors of bone, cartilage, fat, muscle, vascular, and hematopoietic tissues...
s.
Testing for PPB
The most common way to test someone for PPB is to take a biopsyBiopsy
A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...
. Other tests like x-rays, CAT scans, and MRI's can suggest that cancer is present, but only an examination of a piece of the tumor can make a definite diagnosis.
External links
- Pleuropulmonary Blastoma Bulletin Board
- Pleuropulmonary Blastoma Family Support Group
- Pleuropulmonary blastoma images at humpath.com
Treating
Treating PPB depends on the size and location of the tumorTumor
A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...
, whether the cancer
Cancer
Cancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...
has spread, and the child's overall health. Surgery is the main treatment for PPB. The main goal of surgery is to remove the tumor
Tumor
A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...
. If the tumor is too large to be completely removed, or if it's not possible to completely remove the tumor, surgery may be performed after chemotherapy
Chemotherapy
Chemotherapy is the treatment of cancer with an antineoplastic drug or with a combination of such drugs into a standardized treatment regimen....
. Because PPB can return after treatment, regular screening for possible recurrence should continue for 48 to 60 months, after diagnosis.