Pleuropulmonary blastoma: Difference between revisions

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__NOTOC__
#REDIRECT [[Sarcomatoid carcinoma of the lung pathophysiology#Microscopic Pathology]]
{{SI}}
{{CMG}}; {{AE}} {{Faizan}}, {{MV}}
 
{{SK}} Pulmonary blastoma; PPB
==Overview==
'''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>
 
==Historical Perspective==
*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>
 
==Classification==
*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>
:* Type I: multicystic lesions
:* Type II: thickened areas ([[nodule]]s) 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.
 
==Pathophysiology==
*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>
*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>
*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>
:*Extrapumonary mass
:*Attachment to the [[parietal pleura]]
*On microscopic histopathological analysis, characteristic findings of pleuropulmonary blastoma include:
:*[[Epithelial]] component
:*[[Mesenchymal]] component
:*Small, round cells
*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>
:*Positive for [[TTF-1]]
:*Positive for [[vimentin]]
:*Positive for epithelial membrane antigen (EMA)
 
==Causes==
* 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>
:*[[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 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>
:*Intrathoracic [[soft tissue sarcoma]]
:*Large [[bronchogenic cyst]] or lung cyst (for type I)
:*Fetal lung interstitial tumor
 
==Epidemiology and Demographics==
*Pleuropulmonary blastoma is very uncommon.<ref name="radoi"> Radiopedia. Dr. Henry Knipe. http://radiopaedia.org/articles/pleuropulmonary-blastoma Accessed on April 20, 2016 </ref>
 
===Age===
*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.<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>
===Gender===
*Pleuropulmonary blastoma affects men and women equally.
 
===Race===
*There is no racial predilection for pleuropulmonary blastoma
 
==Risk Factors==
*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>
 
== Natural History, Complications and Prognosis==
*The majority of patients with pleuropulmonary blastoma are asymptomatic.
*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>
*Early clinical features include persisting [[Upper respiratory tract infection causes|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.
 
== Diagnosis ==
=== Symptoms ===
*Pleuropulmonary blastoma is usually asymptomatic.
*Symptoms of pleuropulmonary blastoma are often non-specific.
*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>
:*[[Chest pain]]
:*[[Cough]]
:*[[Shortness of breath]]
 
=== Physical Examination ===
*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>
*Physical examination may be remarkable for:
:*Eye anomalies
:*[[Mental retardation]]
:*Skin [[Hypopigmentation skin|hypopigmentation]]
:*Congenital skeletal abnormalities
:*Short stature
:*Premature aging
 
=== Laboratory Findings ===
*There are no specific laboratory findings associated with pleuropulmonary blastoma.
 
===Imaging Findings===
*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]].<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>
 
== Treatment ==
=== Medical Therapy ===
*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>
 
=== Surgery ===
*Surgery is the mainstay of therapy for pleuropulmonary blastoma.
*Complete surgical resection is often most common approach to the treatment of pleuropulmonary blastoma.
 
=== Prevention ===
*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>
 
==References==
{{Reflist|2}}
[[Category:Oncology]]
[[Category:Pulmonology]]
 
{{WS}}
{{WH}}
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Medicine]]
[[Category:Pulmonology]]
[[Category:Surgery]]

Latest revision as of 17:46, 19 February 2019