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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
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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>
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| ==Historical Perspective==
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| *Pleuropulmonary blastoma was first discovered by Manivel in 1988.
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| ==Classification==
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| *Pleuropulmonary blastoma may be classified into 3 groups:
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| :* Type I: multicystic lesions
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| :* Type II: thickened areas (nodules) within this 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, and there is some evidence that 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
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| *The p53 mutations/deletions have been associated with the development of pleuropulmonary blastoma.
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| *On gross pathology, characteristic findings of pleuropulmonary blastoma, include:
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| :*The mass can be extrapulmonary
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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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| ==Causes==
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| * Common causes of pleuropulmonary blastoma, include:
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| :*Trisomy 8
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| :*Trisomy 2
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| :*p53 mutations/deletions
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| ==Differentiating [disease name] from other Diseases==
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| *Pleuropulmonary blastoma must be differentiated from other diseases that cause cough, or reccurent upper respiratory tract infections, such as:
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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 tumour
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| ==Epidemiology and Demographics==
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| *Pleuropulmonary blastoma is very uncommon.
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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.
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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 in the development of pleuropulmonary blastoma.
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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 examinations.
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| *Early clinical features include persisting 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 death.
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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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| == 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:
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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 are well-appearing.
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| *Physical examination may be remarkable for:
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| :*[finding 1]
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| :*[finding 2]
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| :*[finding 3]
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| :*[finding 4]
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| :*[finding 5]
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| :*[finding 6]
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| === Laboratory Findings ===
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| *There are no specific laboratory findings associated with
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| ===Imaging Findings===
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| *CT is the imaging modality of choice for pleuropulmonary blastoma
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| *On conventional radiograph, findings of pleuropulmonary blastoma include:
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| :*[[Pseudo-cardiomegaly]]
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| *On CT, findings of pleuropulmonary blastoma include:
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| == Treatment ==
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| === Medical Therapy ===
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| *There is no treatment for [disease name]; the mainstay of therapy is supportive care.
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|
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| *The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
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| *[Medical therapy 1] acts by [mechanism of action1].
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| *Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
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| === Surgery ===
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| *Surgery is the mainstay of therapy for [disease name].
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| *[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
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| *[Surgical procedure] can only be performed for patients with [disease stage] [disease name].
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| === Prevention ===
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| *There are no primary preventive measures available for [disease name].
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| *Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
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| *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].
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| ==References==
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| {{Reflist|2}}
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| [[Category:Pick One of 28 Approved]]
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