Pleuropulmonary blastoma (PPB) is a rare

cancer Cancer is a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body. These contrast with benign tumors, which do not spread. Possible signs and symptoms include a lump, abnormal b ...

originating in the

lung The lungs are the primary organs of the respiratory system in humans and most other animals, including some snails and a small number of fish. In mammals and most other vertebrates, two lungs are located near the backbone on either side of t ...

pleural cavity The pleural cavity, pleural space, or interpleural space is the potential space between the pleurae of the pleural sac that surrounds each lung. A small amount of serous pleural fluid is maintained in the pleural cavity to enable lubrication bet ...

. 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 A carcinoid (also carcinoid tumor) is a slow-growing type of neuroendocrine tumor originating in the cells of the neuroendocrine system. In some cases, metastasis may occur. Carcinoid tumors of the midgut (jejunum, ileum, appendix, and cecum) a ...

were the most common primary tumors (83% of the 204 children had secondary tumors

spread Spread may refer to: Places * Spread, West Virginia Arts, entertainment, and media * ''Spread'' (film), a 2009 film. * ''$pread'', a quarterly magazine by and for sex workers * "Spread", a song by OutKast from their 2003 album ''Speakerboxxx/T ...

from cancers elsewhere in the body). Pleuropulmonary blastoma is regarded as

malignant Malignancy () is the tendency of a medical condition to become progressively worse. Malignancy is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous ''benign'' tumor in that a malignancy is not s ...

. The male:female ratio is approximately one.

Signs and symptoms

Symptoms may include

cough A cough is a sudden expulsion of air through the large breathing passages that can help clear them of fluids, irritants, foreign particles and microbes. As a protective reflex, coughing can be repetitive with the cough reflex following three ph ...

ing, an

upper respiratory tract infection An upper respiratory tract infection (URTI) is an illness caused by an acute infection, which involves the upper respiratory tract, including the nose, sinuses, pharynx, larynx or trachea. This commonly includes nasal obstruction, sore thro ...

shortness of breath Shortness of breath (SOB), also medically known as dyspnea (in AmE) or dyspnoea (in BrE), is an uncomfortable feeling of not being able to breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing disc ...

, 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

, until more test are taken and they show that there is no infection. Another symptom is

pneumothorax A pneumothorax is an abnormal collection of air in the pleural space between the lung and the chest wall. Symptoms typically include sudden onset of sharp, one-sided chest pain and shortness of breath. In a minority of cases, a one-way valve i ...

Genetics

A number of PPBs have shown trisomy 8 (17 out of 23 cases studied per the PPB registry). Trisomy 2 and p53 mutations/deletions have also been described. An association with mutations in the

DICER1 Dicer, also known as endoribonuclease Dicer or helicase with RNase motif, is an enzyme that in humans is encoded by the gene. Being part of the RNase III family, Dicer cleaves double-stranded RNA (dsRNA) and pre-microRNA (pre-miRNA) into short d ...

gene has been reported.Cai S, Wang X, Zhao W, Fu L, Ma X, Peng X (2017) DICER1 mutations in twelve Chinese patients with pleuropulmonary blastoma. Sci China Life Sci doi: 10.1007/s11427-017-9081-x Mutations in this gene are found in 2/3 cases.

Diagnosis

The most common way to test someone for PPB is to take a

biopsy A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, or an interventional cardiologist. The process involves extraction of sample cells or tissues for examination to determine the presence or extent of a dise ...

. Other tests like

x-rays An X-ray, or, much less commonly, X-radiation, is a penetrating form of high-energy electromagnetic radiation. Most X-rays have a wavelength ranging from 10 picometers to 10 nanometers, corresponding to frequencies in the range 30&nbs ...

, 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.

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 A cyst is a closed sac, having a distinct envelope and division compared with the nearby tissue. Hence, it is a cluster of cells that have grouped together to form a sac (like the manner in which water molecules group together to form a bubble) ...

, 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 A sarcoma is a malignant tumor, a type of cancer that arises from transformed cells of mesenchymal ( connective tissue) origin. Connective tissue is a broad term that includes bone, cartilage, fat, vascular, or hematopoietic tissues, and sar ...

Treatment

Treating PPB depends on the size and location of the

tumor A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...

, whether 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

. 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 (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs (chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemotherap ...

. Because PPB can return after treatment, regular screening for possible recurrence should continue for 48 to 60 months, after diagnosis.

History

Pleuropulmonary blastoma was first described in 1988. An international registry has been established.

References

External links

{{Respiratory and intrathoracic neoplasia Rare cancers Lung cancer Pediatric cancers