Pleuropulmonary blastoma: Difference between revisions
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==Overview== | ==Overview== | ||
'''Pleuropulmonary blastoma''' ('''PPB''') is a rare | '''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== | ==Historical Perspective== | ||
*Pleuropulmonary blastoma was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event]. | *Pleuropulmonary blastoma was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event]. | ||
==Classification== | ==Classification== | ||
*Pleuropulmonary blastoma may be classified into 3 groups: | *Pleuropulmonary blastoma may be classified into 3 groups: | ||
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==Pathophysiology== | ==Pathophysiology== | ||
*The pathogenesis of | *The pathogenesis of pleuropulmonary blastoma is characterized by | ||
*The | *The p53 mutations/deletions have been associated with the development of pleuropulmonary blastoma. | ||
*On gross pathology, characteristic findings of pleuropulmonary blastoma, include: | *On gross pathology, characteristic findings of pleuropulmonary blastoma, include: | ||
*On microscopic histopathological analysis, characteristic findings of pleuropulmonary blastoma, include: | *On microscopic histopathological analysis, characteristic findings of pleuropulmonary blastoma, include: | ||
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==Causes== | ==Causes== | ||
* Common causes of pleuropulmonary blastoma, include: | * Common causes of pleuropulmonary blastoma, include: | ||
:*Trisomy 8 | |||
:*Trisomy 2 | |||
:*p53 mutations/deletions | |||
==Differentiating [disease name] from other Diseases== | ==Differentiating [disease name] from other Diseases== | ||
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===Age=== | ===Age=== | ||
*Pleuropulmonary blastoma is more commonly observed among patients between 0-2 years old. | |||
*Pleuropulmonary blastoma is more commonly observed among patients | |||
*Pleuropulmonary blastoma is more commonly observed among infants and children. | *Pleuropulmonary blastoma is more commonly observed among infants and children. | ||
*Pleuropulmonary blastoma is less commonly observed among adults. | *Pleuropulmonary blastoma is less commonly observed among adults. | ||
Revision as of 18:55, 20 April 2016
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
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]
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:
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:
- 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
- ↑ 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.