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
==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>
 
==Historical Perspective==
*Pleuropulmonary blastoma was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
 
==Classification==
*Pleuropulmonary blastoma may be classified into 3 groups:
:* Type I: multicystic lesions
:* Type II: thickened areas (nodules) within this 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, and there is some evidence that not all type I PPB will progress to types II and III.
==Pathophysiology==
*The pathogenesis of pleuropulmonary blastoma is characterized by
*The p53 mutations/deletions have been associated with the development of pleuropulmonary blastoma.
*On gross pathology, characteristic findings of pleuropulmonary blastoma, include:
*On microscopic histopathological analysis, characteristic findings of pleuropulmonary blastoma, include:
==Causes==
* Common causes of pleuropulmonary blastoma, include:
:*Trisomy 8
:*Trisomy 2
:*p53 mutations/deletions
 
==Differentiating [disease name] from other Diseases==
*Pleuropulmonary blastoma must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
:*[Differential dx1]
:*[Differential dx2]
:*[Differential dx3]
==Epidemiology and Demographics==
* The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
* In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].
===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.
===Gender===
*Pleuropulmonary blastoma affects men and women equally.
*[Gender 1] are more commonly affected with [disease name] than [gender 2].
* The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
===Race===
*There is no racial predilection for  pleuropulmonary blastoma
==Risk Factors==
*There are no risk factors associated in the development of pleuropulmonary blastoma.
== Natural History, Complications and Prognosis==
*The majority of patients with pleuropulmonary blastoma remain asymptomatic.
*Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
*If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
*Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
*Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].
== Diagnosis ==
 
=== Symptoms ===
*Pleuropulmonary blastoma is usually asymptomatic.
*Symptoms of pleuropulmonary blastoma are often non-specific.
*Symptoms of pleuropulmonary blastoma may include the following:
:*[[Chest pain]]
:*[[Cough]]
:*[[Shortness of breath]]
 
=== Physical Examination ===
*Patients with pleuropulmonary blastoma usually are well-appearing.
*Physical examination may be remarkable for:
:*[finding 1]
:*[finding 2]
:*[finding 3]
:*[finding 4]
:*[finding 5]
:*[finding 6]
=== Laboratory Findings ===
*There are no specific laboratory findings associated with
 
===Imaging Findings===
*CT is the imaging modality of choice for pleuropulmonary blastoma
*On conventional radiograph, findings of pleuropulmonary blastoma  include:
:*[[Pseudo-cardiomegaly]]
*On CT, findings of pleuropulmonary blastoma  include:
 
== Treatment ==
=== Medical Therapy ===
*There is no treatment for [disease name]; the mainstay of therapy is supportive care.
*The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
=== Surgery ===
*Surgery is the mainstay of therapy for [disease name].
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
*[Surgical procedure] can only be performed for patients with [disease stage] [disease name].
=== Prevention ===
*There are no primary preventive measures available for [disease name].
*Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
 
*Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].
==References==
{{Reflist|2}}
[[Category:Pick One of 28 Approved]]

Latest revision as of 17:46, 19 February 2019