WikiDoc Resources for Pleuropulmonary blastoma
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Synonyms and Keywords: Pulmonary blastoma; PPB
Pleuropulmonary blastoma (PPB) is a rare intrathoracic malignancy originating in the lung or pleural cavity. Pleuropulmonary blastoma occurs most often in infants and young children. The pathogenesis of pleuropulmonary blastoma is characterized by resembling immature lung tissue. The p53 mutations/deletions have been associated with the development of pleuropulmonary blastoma. Pleuropulmonary blastoma may be classified into 3 groups: type I, II, III. Common causes of pleuropulmonary blastoma include trisomy 8, trisomy 2, and p53 mutations/deletions (e.g., Li-Fraumeni syndrome). On gross pathology, characteristic findings of pleuropulmonary blastoma include extra-pulmonary location, and attachment to the parietal pleura. Pleuropulmonary blastoma is more commonly observed among patients between 0-2 years old. Complete surgical resection is often most common approach to the treatment of pleuropulmonary blastoma.
- Pleuropulmonary blastoma was first discovered by Dr. Juan C. Manivel, MD in 1988.
- Pleuropulmonary blastoma may be classified into 3 groups:
- Type I: multicystic lesions
- Type II: thickened areas (nodules) within cystic lesions
- Type III: solid masses
- Type I PPB is made up of mostly cysts, and may be hard to distinguish from benign lung cysts; not all type I PPB will progress to types II and III.
- The pathogenesis of pleuropulmonary blastoma is characterized by resembling immature lung tissue.
- The p53 and DICER-1 gene mutations/deletions have been associated with the development of pleuropulmonary blastoma.
- On gross pathology, characteristic findings of pleuropulmonary blastoma include:
- Extrapumonary mass
- Attachment to the parietal pleura
- On microscopic histopathological analysis, characteristic findings of pleuropulmonary blastoma include:
- On inmunohistochemistry analysis, characteristic findings of pleuropulmonary blastoma include:
- Common causes of pleuropulmonary blastoma include:
- Trisomy 8
- Trisomy 2
- p53 mutations/deletions
Differentiating Pleuropulmonary Blastoma from Other Diseases
- Pleuropulmonary blastoma must be differentiated from other diseases that cause cough or recurrent upper respiratory tract infections, such as:
Epidemiology and Demographics
- Pleuropulmonary blastoma is very uncommon.
- Pleuropulmonary blastoma is more commonly observed among patients between 0-2 years old.
- Pleuropulmonary blastoma is more commonly observed among infants and children.
- Pleuropulmonary blastoma is less commonly observed among adults.
- Pleuropulmonary blastoma affects men and women equally.
- There is no racial predilection for pleuropulmonary blastoma
- There are no risk factors associated with the development of pleuropulmonary blastoma.
Natural History, Complications and Prognosis
- The majority of patients with pleuropulmonary blastoma are asymptomatic.
- Pleuropulmonary blastoma is usually an incidental finding during routine examination.
- Early clinical features include persisting upper respiratory tract infection, coughing, and shortness of breath.
- If left untreated, the majority of patients with may progress to develop acute respiratory distress syndrome.
- Common complications of pleuropulmonary blastoma include respiratory failure, pneumonia, or mortality.
- Prognosis is generally poor, and the 5-year survival rate of patients with pleuropulmonary blastoma is approximately 15%.
- Pleuropulmonary blastomas larger than 5 cm have a worse prognosis.
- Pleuropulmonary blastoma is usually asymptomatic.
- Symptoms of pleuropulmonary blastoma are often non-specific.
- Symptoms of pleuropulmonary blastoma may include the following:
- Patients with pleuropulmonary blastoma usually have dysmorphic facies.
- Physical examination may be remarkable for:
- There are no specific laboratory findings associated with pleuropulmonary blastoma.
- CT is the imaging modality of choice for pleuropulmonary blastoma. On CT, findings of pleuropulmonary blastoma may include:
- Large mass in the thorax with solid mixed cystic heterogeneous low attenuation
- Pleural effusion
- Contralateral mediastinal shift
- Lack of chest wall invasion
- On conventional radiograph, findings of pleuropulmonary blastoma may include pseudo-cardiomegaly.
- There is no treatment for pleuropulmonary blastoma; the mainstay of therapy is supportive care.
- Surgery is the mainstay of therapy for pleuropulmonary blastoma.
- Complete surgical resection is often most common approach to the treatment of pleuropulmonary blastoma.
- There are no primary preventive measures available for pleuropulmonary blastoma.
- Indolfi P, Casale F, Carli M, et al. (September 2000). "Pleuropulmonary blastoma: management and prognosis of 11 cases". Cancer. 89 (6): 1396–401. doi:10.1002/1097-0142(20000915)89:6<1396::AID-CNCR25>3.0.CO;2-2. PMID 11002236.
- Radiopedia. Dr. Henry Knipe. http://radiopaedia.org/articles/pleuropulmonary-blastoma Accessed on April 20, 2016
- Manivel JC, Priest JR, Watterson J, Steiner M, Woods WG, Wick MR; et al. (1988). "Pleuropulmonary blastoma. The so-called pulmonary blastoma of childhood". Cancer. 62 (8): 1516–26. PMID 3048630.
- Dehner LP (1994). "Pleuropulmonary blastoma is THE pulmonary blastoma of childhood". Semin Diagn Pathol. 11 (2): 144–51. PMID 7809508.