Pleuropulmonary blastoma: Difference between revisions
Jump to navigation
Jump to search
m (Categories) |
|||
| Line 1: | Line 1: | ||
__NOTOC__ | __NOTOC__ | ||
{{SI}} | {{SI}} | ||
{{CMG}} {{AE}}{{Faizan}} {{MV}} | {{CMG}}; {{AE}} {{Faizan}}, {{MV}} | ||
{{SK}} Pulmonary blastoma; PPB | {{SK}} Pulmonary blastoma; PPB | ||
==Overview== | ==Overview== | ||
'''Pleuropulmonary blastoma''' ('''PPB''') is a rare intrathoracic malignancy originating in the [[lung]] or [[pleural cavity]]. | '''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> | ||
==Historical Perspective== | ==Historical Perspective== | ||
| Line 14: | Line 14: | ||
*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> | *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 I: multicystic lesions | ||
:* Type II: thickened areas ( | :* Type II: thickened areas ([[nodule]]s) within cystic lesions | ||
:* Type III: solid masses | :* Type III: solid masses | ||
*Type I PPB is made up of mostly [[cysts]], and may be hard to distinguish from benign lung cysts | *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== | ==Pathophysiology== | ||
| Line 116: | Line 116: | ||
{{Reflist|2}} | {{Reflist|2}} | ||
[[Category: | [[Category:Oncology]] | ||
[[Category:Pulmonology]] | |||
{{WS}} | |||
{{WH}} | |||
Revision as of 20:38, 27 July 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; 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 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 Manivel in 1988.
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:[3]
- The mass can be extrapulmonary
- 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:[2]
- Positive for TTF-1
- Positive for vimentin
- Positive for EMA
Causes
- Common causes of pleuropulmonary blastoma, include:[2]
- 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 reccurent upper respiratory tract infections, such as:[3]
- Intrathoracic soft tissue sarcoma
- Large bronchogenic cyst or lung cyst (for type I)
- Fetal lung interstitial tumour
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.[3]
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 in 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 examinations.[3]
- 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 death.
- 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:[3]
Physical Examination
- Patients with pleuropulmonary blastoma usually have dysmorphic facies.[3]
- Physical examination may be remarkable for:
- Eye anomalies
- Mental retardation
- 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 conventional radiograph, findings of pleuropulmonary blastoma include:[3]
- 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.[3]
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.[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.
- ↑ 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
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 3.8 Dehner LP (1994). "Pleuropulmonary blastoma is THE pulmonary blastoma of childhood". Semin Diagn Pathol. 11 (2): 144–51. PMID 7809508.