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| __NOTOC__
| | #REDIRECT [[Sarcomatoid carcinoma of the lung pathophysiology#Microscopic Pathology]] |
| {{SI}}
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| {{CMG}}; {{AE}} {{Faizan}}, {{MV}}
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| {{SK}} Pulmonary blastoma; PPB
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| ==Overview==
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| '''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.<ref name="pmid11002236">{{cite journal |vauthors=Indolfi P, Casale F, Carli M, etal |title=Pleuropulmonary blastoma: management and prognosis of 11 cases |journal=Cancer |volume=89 |issue=6 |pages=1396–401 |date=September 2000 |pmid=11002236|doi=10.1002/1097-0142(20000915)89:6<1396::AID-CNCR25>3.0.CO;2-2}}</ref> 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|p53 mutation]]s/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.<ref name="radoi"> Radiopedia. Dr. Henry Knipe. http://radiopaedia.org/articles/pleuropulmonary-blastoma Accessed on April 20, 2016 </ref>
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| ==Historical Perspective==
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| *Pleuropulmonary blastoma was first discovered by Dr. Juan C. Manivel, MD in 1988.<ref name="pmid3048630">{{cite journal| author=Manivel JC, Priest JR, Watterson J, Steiner M, Woods WG, Wick MR et al.| title=Pleuropulmonary blastoma. The so-called pulmonary blastoma of childhood. | journal=Cancer | year= 1988 | volume= 62 | issue= 8 | pages= 1516-26 | pmid=3048630 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3048630 }} </ref>
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| ==Classification==
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| *Pleuropulmonary blastoma may be classified into 3 groups:<ref name="radoi"> Radiopedia. Dr. Henry Knipe. http://radiopaedia.org/articles/pleuropulmonary-blastoma Accessed on April 20, 2016 </ref>
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| :* Type I: multicystic lesions
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| :* Type II: thickened areas ([[nodule]]s) within cystic lesions
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| :* Type III: solid masses
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| *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.
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| ==Pathophysiology==
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| *The pathogenesis of pleuropulmonary blastoma is characterized by resembling immature lung tissue.<ref name="radoi"> Radiopedia. Dr. Henry Knipe. http://radiopaedia.org/articles/pleuropulmonary-blastoma Accessed on April 20, 2016 </ref>
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| *The p53 and [[DICER1|DICER-1]] gene mutations/deletions have been associated with the development of pleuropulmonary blastoma.<ref name="radoi"> Radiopedia. Dr. Henry Knipe. http://radiopaedia.org/articles/pleuropulmonary-blastoma Accessed on April 20, 2016 </ref>
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| *On gross pathology, characteristic findings of pleuropulmonary blastoma include:<ref name="pmid7809508">{{cite journal |vauthors=Dehner LP |title=Pleuropulmonary blastoma is THE pulmonary blastoma of childhood |journal=Semin Diagn Pathol |volume=11 |issue=2 |pages=144–51 |year=1994 |pmid=7809508 |doi= |url=}}</ref>
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| :*Extrapumonary mass
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| :*Attachment to the [[parietal pleura]]
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| *On microscopic histopathological analysis, characteristic findings of pleuropulmonary blastoma include:
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| :*[[Epithelial]] component
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| :*[[Mesenchymal]] component
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| :*Small, round cells
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| *On inmunohistochemistry analysis, characteristic findings of pleuropulmonary blastoma include:<ref name="radoi"> Radiopedia. Dr. Henry Knipe. http://radiopaedia.org/articles/pleuropulmonary-blastoma Accessed on April 20, 2016 </ref>
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| :*Positive for [[TTF-1]]
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| :*Positive for [[vimentin]]
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| :*Positive for epithelial membrane antigen (EMA)
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| ==Causes==
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| * Common causes of pleuropulmonary blastoma include:<ref name="radoi"> Radiopedia. Dr. Henry Knipe. http://radiopaedia.org/articles/pleuropulmonary-blastoma Accessed on April 20, 2016 </ref>
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| :*[[Trisomy 8]]
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| :*Trisomy 2
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| :*p53 mutations/deletions
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| ==Differentiating Pleuropulmonary Blastoma from Other Diseases==
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| *Pleuropulmonary blastoma must be differentiated from other diseases that cause [[cough]] or recurrent [[upper respiratory tract infection]]s, such as:<ref name="pmid7809508">{{cite journal |vauthors=Dehner LP |title=Pleuropulmonary blastoma is THE pulmonary blastoma of childhood |journal=Semin Diagn Pathol |volume=11 |issue=2 |pages=144–51 |year=1994 |pmid=7809508 |doi= |url=}}</ref>
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| :*Intrathoracic [[soft tissue sarcoma]]
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| :*Large [[bronchogenic cyst]] or lung cyst (for type I)
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| :*Fetal lung interstitial tumor
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| ==Epidemiology and Demographics==
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| *Pleuropulmonary blastoma is very uncommon.<ref name="radoi"> Radiopedia. Dr. Henry Knipe. http://radiopaedia.org/articles/pleuropulmonary-blastoma Accessed on April 20, 2016 </ref>
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| ===Age===
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| *Pleuropulmonary blastoma is more commonly observed among patients between 0-2 years old.
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| *Pleuropulmonary blastoma is more commonly observed among infants and children.
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| *Pleuropulmonary blastoma is less commonly observed among adults.<ref name="pmid7809508">{{cite journal |vauthors=Dehner LP |title=Pleuropulmonary blastoma is THE pulmonary blastoma of childhood |journal=Semin Diagn Pathol |volume=11 |issue=2 |pages=144–51 |year=1994 |pmid=7809508 |doi= |url=}}</ref>
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| ===Gender===
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| *Pleuropulmonary blastoma affects men and women equally.
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| ===Race===
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| *There is no racial predilection for pleuropulmonary blastoma
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| ==Risk Factors==
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| *There are no risk factors associated with the development of pleuropulmonary blastoma.<ref name="radoi"> Radiopedia. Dr. Henry Knipe. http://radiopaedia.org/articles/pleuropulmonary-blastoma Accessed on April 20, 2016 </ref>
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| == Natural History, Complications and Prognosis==
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| *The majority of patients with pleuropulmonary blastoma are asymptomatic.
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| *Pleuropulmonary blastoma is usually an incidental finding during routine examination.<ref name="pmid7809508">{{cite journal |vauthors=Dehner LP |title=Pleuropulmonary blastoma is THE pulmonary blastoma of childhood |journal=Semin Diagn Pathol |volume=11 |issue=2 |pages=144–51 |year=1994 |pmid=7809508 |doi= |url=}}</ref>
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| *Early clinical features include persisting [[Upper respiratory tract infection causes|upper respiratory tract infection]], [[coughing]], and [[shortness of breath]].
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| *If left untreated, the majority of patients with may progress to develop [[acute respiratory distress syndrome]].
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| *Common complications of pleuropulmonary blastoma include [[respiratory failure]], [[pneumonia]], or [[mortality]].
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| *Prognosis is generally poor, and the 5-year survival rate of patients with pleuropulmonary blastoma is approximately 15%.
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| *Pleuropulmonary blastomas larger than 5 cm have a worse prognosis.
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| == Diagnosis ==
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| === Symptoms ===
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| *Pleuropulmonary blastoma is usually asymptomatic.
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| *Symptoms of pleuropulmonary blastoma are often non-specific.
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| *Symptoms of pleuropulmonary blastoma may include the following:<ref name="pmid7809508">{{cite journal |vauthors=Dehner LP |title=Pleuropulmonary blastoma is THE pulmonary blastoma of childhood |journal=Semin Diagn Pathol |volume=11 |issue=2 |pages=144–51 |year=1994 |pmid=7809508 |doi= |url=}}</ref>
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| :*[[Chest pain]]
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| :*[[Cough]]
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| :*[[Shortness of breath]]
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| === Physical Examination ===
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| *Patients with pleuropulmonary blastoma usually have dysmorphic facies.<ref name="pmid7809508">{{cite journal |vauthors=Dehner LP |title=Pleuropulmonary blastoma is THE pulmonary blastoma of childhood |journal=Semin Diagn Pathol |volume=11 |issue=2 |pages=144–51 |year=1994 |pmid=7809508 |doi= |url=}}</ref>
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| *Physical examination may be remarkable for:
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| :*Eye anomalies
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| :*[[Mental retardation]]
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| :*Skin [[Hypopigmentation skin|hypopigmentation]]
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| :*Congenital skeletal abnormalities
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| :*Short stature
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| :*Premature aging
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| === Laboratory Findings ===
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| *There are no specific laboratory findings associated with pleuropulmonary blastoma.
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| ===Imaging Findings===
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| *CT is the imaging modality of choice for pleuropulmonary blastoma. On CT, findings of pleuropulmonary blastoma may include:
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| :*Large mass in the thorax with solid mixed cystic heterogeneous low attenuation
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| :*[[Pleural effusion]]
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| :*Contralateral mediastinal shift
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| :*Lack of chest wall invasion
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| *On conventional radiograph, findings of pleuropulmonary blastoma may include [[pseudo-cardiomegaly]].<ref name="pmid7809508">{{cite journal |vauthors=Dehner LP |title=Pleuropulmonary blastoma is THE pulmonary blastoma of childhood |journal=Semin Diagn Pathol |volume=11 |issue=2 |pages=144–51 |year=1994 |pmid=7809508 |doi= |url=}}</ref>
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| == Treatment ==
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| === Medical Therapy ===
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| *There is no treatment for pleuropulmonary blastoma; the mainstay of therapy is supportive care.<ref name="pmid7809508">{{cite journal |vauthors=Dehner LP |title=Pleuropulmonary blastoma is THE pulmonary blastoma of childhood |journal=Semin Diagn Pathol |volume=11 |issue=2 |pages=144–51 |year=1994 |pmid=7809508 |doi= |url=}}</ref>
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| === Surgery ===
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| *Surgery is the mainstay of therapy for pleuropulmonary blastoma.
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| *Complete surgical resection is often most common approach to the treatment of pleuropulmonary blastoma.
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| === Prevention ===
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| *There are no primary preventive measures available for pleuropulmonary blastoma.<ref name="pmid7809508">{{cite journal |vauthors=Dehner LP |title=Pleuropulmonary blastoma is THE pulmonary blastoma of childhood |journal=Semin Diagn Pathol |volume=11 |issue=2 |pages=144–51 |year=1994 |pmid=7809508 |doi= |url=}}</ref>
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| ==References==
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| {{Reflist|2}}
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| [[Category:Oncology]]
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| [[Category:Pulmonology]]
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| {{WS}}
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| {{WH}}
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| [[Category:Up-To-Date]]
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| [[Category:Oncology]]
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| [[Category:Medicine]]
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| [[Category:Pulmonology]]
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| [[Category:Surgery]]
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