Pleuropulmonary blastoma: Difference between revisions

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==Overview==
==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 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>
'''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==
==Historical Perspective==
*Pleuropulmonary blastoma was first discovered by Manivel in 1988.
*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==
==Classification==
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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>
*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>
*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>
*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>
:*The mass can be extrapulmonary
:*Extrapumonary mass
:*Attachment to the parietal pleura
:*Attachment to the [[parietal pleura]]
*On microscopic histopathological analysis, characteristic findings of pleuropulmonary blastoma, include:
*On microscopic histopathological analysis, characteristic findings of pleuropulmonary blastoma include:
:*Epithelial component
:*[[Epithelial]] component
:*Mesenchymal component
:*[[Mesenchymal]] component
:*Small round cells
:*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>
*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 [[TTF-1]]
:*Positive for vimentin  
:*Positive for [[vimentin]]
:*Positive for EMA
:*Positive for epithelial membrane antigen (EMA)


==Causes==
==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>
* 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 8]]
:*Trisomy 2  
:*Trisomy 2  
:*p53 mutations/deletions
:*p53 mutations/deletions


==Differentiating Pleuropulmonary Blastoma from Other Diseases==
==Differentiating Pleuropulmonary Blastoma from Other Diseases==
*Pleuropulmonary blastoma must be differentiated from other diseases that cause cough, or reccurent upper respiratory tract infections, 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>
*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
:*Intrathoracic [[soft tissue sarcoma]]
:*Large bronchogenic cyst or lung cyst (for type I)
:*Large [[bronchogenic cyst]] or lung cyst (for type I)
:*Fetal lung interstitial tumour
:*Fetal lung interstitial tumor


==Epidemiology and Demographics==
==Epidemiology and Demographics==
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===Race===
===Race===
*There is no racial predilection for pleuropulmonary blastoma
*There is no racial predilection for pleuropulmonary blastoma


==Risk Factors==
==Risk Factors==
*There are no risk factors associated in the development of pleuropulmonary blastoma.<ref name="radoi"> Radiopedia. Dr. Henry Knipe. http://radiopaedia.org/articles/pleuropulmonary-blastoma Accessed on April 20, 2016 </ref>  
*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==
== Natural History, Complications and Prognosis==
*The majority of patients with pleuropulmonary blastoma are asymptomatic.  
*The majority of patients with pleuropulmonary blastoma are asymptomatic.  
*Pleuropulmonary blastoma is usually an incidental finding during routine examinations.<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>
*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]].  
*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.  
*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 death.  
*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%
*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 blastomas larger than 5 cm have a worse prognosis.


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===Imaging Findings===
===Imaging Findings===
*CT is the imaging modality of choice for pleuropulmonary blastoma  
*CT is the imaging modality of choice for pleuropulmonary blastoma. On CT, findings of pleuropulmonary blastoma may include:
*On conventional radiograph, 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>
:*[[Pseudo-cardiomegaly]]
*On CT, findings of pleuropulmonary blastoma may include:
:*Large mass in the thorax with solid mixed cystic heterogeneous low attenuation
:*Large mass in the thorax with solid mixed cystic heterogeneous low attenuation
:*[[Pleural effusion]]
:*[[Pleural effusion]]
:*Contralateral mediastinal shift
:*Contralateral mediastinal shift
:*Lack of chest wall invasion  
:*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 ==
== Treatment ==
=== Medical Therapy ===
=== 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>
*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 ===
*Surgery is the mainstay of therapy for pleuropulmonary blastoma.
*Surgery is the mainstay of therapy for pleuropulmonary blastoma.
*Complete surgical resection is often most common approach to the treatment of pleuropulmonary blastoma
*Complete surgical resection is often most common approach to the treatment of pleuropulmonary blastoma.


=== Prevention ===
=== Prevention ===
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[[Category:Oncology]]
[[Category:Oncology]]
[[Category:Pulmonology]]
[[Category:Pulmonology]]
[[Category:FinalQCRequired]]


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Revision as of 20:58, 27 July 2016

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Faizan Sheraz, M.D. [2], Maria Fernanda Villarreal, M.D. [3]

Synonyms and keywords: 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.[1] 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.[2]

Historical Perspective

  • Pleuropulmonary blastoma was first discovered by Dr. Juan C. Manivel, MD in 1988.[3]

Classification

  • Pleuropulmonary blastoma may be classified into 3 groups:[2]
  • 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.

Pathophysiology

  • The pathogenesis of pleuropulmonary blastoma is characterized by resembling immature lung tissue.[2]
  • The p53 and DICER-1 gene mutations/deletions have been associated with the development of pleuropulmonary blastoma.[2]
  • On gross pathology, characteristic findings of pleuropulmonary blastoma include:[4]
  • On microscopic histopathological analysis, characteristic findings of pleuropulmonary blastoma include:
  • On inmunohistochemistry analysis, characteristic findings of pleuropulmonary blastoma include:[2]
  • Positive for TTF-1
  • Positive for vimentin
  • Positive for epithelial membrane antigen (EMA)

Causes

  • Common causes of pleuropulmonary blastoma include:[2]

Differentiating Pleuropulmonary Blastoma from Other Diseases

Epidemiology and Demographics

  • Pleuropulmonary blastoma is very uncommon.[2]

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.[4]

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.[2]

Natural History, Complications and Prognosis

  • The majority of patients with pleuropulmonary blastoma are asymptomatic.
  • Pleuropulmonary blastoma is usually an incidental finding during routine examination.[4]
  • 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.

Diagnosis

Symptoms

  • Pleuropulmonary blastoma is usually asymptomatic.
  • Symptoms of pleuropulmonary blastoma are often non-specific.
  • Symptoms of pleuropulmonary blastoma may include the following:[4]

Physical Examination

  • Patients with pleuropulmonary blastoma usually have dysmorphic facies.[4]
  • Physical examination may be remarkable for:

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

Treatment

Medical Therapy

  • There is no treatment for pleuropulmonary blastoma; the mainstay of therapy is supportive care.[4]

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.[4]

References

  1. 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.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 Radiopedia. Dr. Henry Knipe. http://radiopaedia.org/articles/pleuropulmonary-blastoma Accessed on April 20, 2016
  3. 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.
  4. 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 4.8 Dehner LP (1994). "Pleuropulmonary blastoma is THE pulmonary blastoma of childhood". Semin Diagn Pathol. 11 (2): 144–51. PMID 7809508.

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