Difference between revisions of "Pulmonary blastoma"

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'''Pulmonary blastoma''' is a very rare tumour of the [[lung]].
'''Pulmonary blastoma''' is a very rare tumour of the [[lung]]. These tumours are often lumped into the ''[[pulmonary sarcomatoid carcinomas]]'' group.<ref name=pmid19124452>{{Cite journal  | last1 = Pelosi | first1 = G. | last2 = Sonzogni | first2 = A. | last3 = De Pas | first3 = T. | last4 = Galetta | first4 = D. | last5 = Veronesi | first5 = G. | last6 = Spaggiari | first6 = L. | last7 = Manzotti | first7 = M. | last8 = Fumagalli | first8 = C. | last9 = Bresaola | first9 = E. | title = Review article: pulmonary sarcomatoid carcinomas: a practical overview. | journal = Int J Surg Pathol | volume = 18 | issue = 2 | pages = 103-20 | month = Apr | year = 2010 | doi = 10.1177/1066896908330049 | PMID = 19124452 }}</ref>


The article deals with ''well-differentiated fetal adenocarcinoma'' and ''classic biphasic pulmonary blastoma''.
[[Pleuropulmonary blastoma]] is dealt with in a separate article.
==General==
==General==
*Very rare ~ 100 cases in the literature.<ref name=pmid23049565 >{{Cite journal  | last1 = Alahwal | first1 = MS. | last2 = Maniyar | first2 = IH. | last3 = Saleem | first3 = F. | last4 = Alshiekh | first4 = M. | title = Pulmonary blastoma: a rare primary lung malignancy. | journal = Case Rep Med | volume = 2012 | issue =  | pages = 471613 | month =  | year = 2012 | doi = 10.1155/2012/471613 | PMID = 23049565 | PMC = 3461274 }}</ref>
*Very rare ~ 100 cases in the literature.<ref name=pmid23049565 >{{Cite journal  | last1 = Alahwal | first1 = MS. | last2 = Maniyar | first2 = IH. | last3 = Saleem | first3 = F. | last4 = Alshiekh | first4 = M. | title = Pulmonary blastoma: a rare primary lung malignancy. | journal = Case Rep Med | volume = 2012 | issue =  | pages = 471613 | month =  | year = 2012 | doi = 10.1155/2012/471613 | PMID = 23049565 | PMC = 3461274 }}</ref>
*Abysmal prognosis - 5-year survival ~ 15%.<ref name=pmid23049565/>
*Abysmal prognosis - 5-year survival ~ 15%.<ref name=pmid23049565/>
*Typical patient <20 years old.<ref name=pmid23427368>{{Cite journal  | last1 = Jethava | first1 = A. | last2 = Dasanu | first2 = CA. | title = Adult biphasic pulmonary blastoma. | journal = Conn Med | volume = 77 | issue = 1 | pages = 19-22 | month = Jan | year = 2013 | doi =  | PMID = 23427368 }}</ref>
*Typical patient <20 years old.
**Adults usu. in 40s.
**Adults usu. in 40s.<ref name=pmid23427368>{{Cite journal  | last1 = Jethava | first1 = A. | last2 = Dasanu | first2 = CA. | title = Adult biphasic pulmonary blastoma. | journal = Conn Med | volume = 77 | issue = 1 | pages = 19-22 | month = Jan | year = 2013 | doi =  | PMID = 23427368 | URL = http://bjr.birjournals.org/content/78/929/437.full }}</ref>
 
*Resemble immature lung tissue.<ref name=pmid16447486>{{Cite journal  | last1 = Schulze | first1 = SM. | last2 = Sbayi | first2 = S. | last3 = Costic | first3 = JT. | last4 = Moser | first4 = RL. | title = A rare case of classic biphasic pulmonary blastoma. | journal = Am Surg | volume = 71 | issue = 12 | pages = 1078-81 | month = Dec | year = 2005 | doi =  | PMID = 16447486 }}</ref>
Subtypes:<ref name=pmid21513998>{{Cite journal  | last1 = Van Loo | first1 = S. | last2 = Boeykens | first2 = E. | last3 = Stappaerts | first3 = I. | last4 = Rutsaert | first4 = R. | title = Classic biphasic pulmonary blastoma: a case report and review of the literature. | journal = Lung Cancer | volume = 73 | issue = 2 | pages = 127-32 | month = Aug | year = 2011 | doi = 10.1016/j.lungcan.2011.03.018 | PMID = 21513998 }}</ref>
===Classification===
Traditionally subdivided into:<ref name=pmid15845939>{{Cite journal  | last1 = Walker | first1 = RI. | last2 = Suvarna | first2 = K. | last3 = Matthews | first3 = S. | title = Case report: pulmonary blastoma: presentation of two atypical cases and review of the literature. | journal = Br J Radiol | volume = 78 | issue = 929 | pages = 437-40 | month = May | year = 2005 | doi = 10.1259/bjr/45172814 | PMID = 15845939 }}</ref>
*Well-differentiated fetal adenocarcinoma (WDFA).
*Well-differentiated fetal adenocarcinoma (WDFA).
*Classic biphasic pulmonary blastoma (CBPB).
*Classic biphasic pulmonary blastoma (CBPB).
*Pleuropulmonary blastoma - considered a separate entity.  
*[[Pleuropulmonary blastoma]].
**Now considered a separate entity.<ref name=pmid21513998>{{Cite journal  | last1 = Van Loo | first1 = S. | last2 = Boeykens | first2 = E. | last3 = Stappaerts | first3 = I. | last4 = Rutsaert | first4 = R. | title = Classic biphasic pulmonary blastoma: a case report and review of the literature. | journal = Lung Cancer | volume = 73 | issue = 2 | pages = 127-32 | month = Aug | year = 2011 | doi = 10.1016/j.lungcan.2011.03.018 | PMID = 21513998 }}</ref>
 
==Gross==
==Gross==
*Lung tumour.
*Lung tumour.
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==Microscopic==
==Microscopic==
Features:
Features:
*Epithelial component:
*Epithelial component.
*Mesenchymal component:
*Mesenchymal component.
**Small round cells.
**Small round cells.


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*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3461274/figure/fig4/ Pulmonary blastoma (nih.gov)].
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3461274/figure/fig4/ Pulmonary blastoma (nih.gov)].
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3461274/figure/fig3/ Pulmonary blastoma (nih.gov)].
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3461274/figure/fig3/ Pulmonary blastoma (nih.gov)].
*[http://path.upmc.edu/cases/case46/dx.html Fetal type adenocarcinoma (upmc.edu)].
*[http://www.surgicalpathologyatlas.com/glfusion/mediagallery/media.php?s=20080802170406269 Pulmonary blastoma (surgicalpathologyatlas.com)].
*[http://www.flickr.com/photos/pulmonary_pathology/5463058190/ Pulmonary blastoma (flickr.com/Yale Rosen)].


==IHC==
==IHC==
Features:<ref>{{Cite journal  | last1 = Zhang | first1 = GQ. | last2 = Fang | first2 = XZ. | last3 = Wang | first3 = BQ. | last4 = Sun | first4 = W. | last5 = Salai | first5 = AD. | title = [Clinicopathological and immunohistochemical features of pulmonary blastoma: analysis of 4 cases and review of the literature]. | journal = Zhonghua Yi Xue Za Zhi | volume = 87 | issue = 15 | pages = 1040-2 | month = Apr | year = 2007 | doi =  | PMID = 17672968 }}</ref>
Features:<ref>{{Cite journal  | last1 = Zhang | first1 = GQ. | last2 = Fang | first2 = XZ. | last3 = Wang | first3 = BQ. | last4 = Sun | first4 = W. | last5 = Salai | first5 = AD. | title = [Clinicopathological and immunohistochemical features of pulmonary blastoma: analysis of 4 cases and review of the literature]. | journal = Zhonghua Yi Xue Za Zhi | volume = 87 | issue = 15 | pages = 1040-2 | month = Apr | year = 2007 | doi =  | PMID = 17672968 }}</ref>
*TTF-1 +ve.
*[[TTF-1]] +ve.
*S-100 -ve.
*S-100 -ve.
*Vimentin -ve.
*Vimentin -ve.{{fact}}
*EMA -ve.
*[[EMA]] -ve.


==See also==
==See also==
Line 40: Line 49:
==References==
==References==
{{Reflist|1}}
{{Reflist|1}}


[[Category:Weird stuff]]
[[Category:Weird stuff]]
[[Category:Diagnosis]]
[[Category:Diagnosis]]

Latest revision as of 04:46, 18 November 2015

Pulmonary blastoma is a very rare tumour of the lung. These tumours are often lumped into the pulmonary sarcomatoid carcinomas group.[1]

The article deals with well-differentiated fetal adenocarcinoma and classic biphasic pulmonary blastoma. Pleuropulmonary blastoma is dealt with in a separate article.

General

  • Very rare ~ 100 cases in the literature.[2]
  • Abysmal prognosis - 5-year survival ~ 15%.[2]
  • Typical patient <20 years old.
    • Adults usu. in 40s.[3]
  • Resemble immature lung tissue.[4]

Classification

Traditionally subdivided into:[5]

  • Well-differentiated fetal adenocarcinoma (WDFA).
  • Classic biphasic pulmonary blastoma (CBPB).
  • Pleuropulmonary blastoma.
    • Now considered a separate entity.[6]

Gross

  • Lung tumour.

Microscopic

Features:

  • Epithelial component.
  • Mesenchymal component.
    • Small round cells.

DDx:

Images

IHC

Features:[7]

See also

References

  1. ↑ Pelosi, G.; Sonzogni, A.; De Pas, T.; Galetta, D.; Veronesi, G.; Spaggiari, L.; Manzotti, M.; Fumagalli, C. et al. (Apr 2010). "Review article: pulmonary sarcomatoid carcinomas: a practical overview.". Int J Surg Pathol 18 (2): 103-20. doi:10.1177/1066896908330049. PMID 19124452.
  2. ↑ 2.0 2.1 Alahwal, MS.; Maniyar, IH.; Saleem, F.; Alshiekh, M. (2012). "Pulmonary blastoma: a rare primary lung malignancy.". Case Rep Med 2012: 471613. doi:10.1155/2012/471613. PMC 3461274. PMID 23049565. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3461274/.
  3. ↑ Jethava, A.; Dasanu, CA. (Jan 2013). "Adult biphasic pulmonary blastoma.". Conn Med 77 (1): 19-22. PMID 23427368.
  4. ↑ Schulze, SM.; Sbayi, S.; Costic, JT.; Moser, RL. (Dec 2005). "A rare case of classic biphasic pulmonary blastoma.". Am Surg 71 (12): 1078-81. PMID 16447486.
  5. ↑ Walker, RI.; Suvarna, K.; Matthews, S. (May 2005). "Case report: pulmonary blastoma: presentation of two atypical cases and review of the literature.". Br J Radiol 78 (929): 437-40. doi:10.1259/bjr/45172814. PMID 15845939.
  6. ↑ Van Loo, S.; Boeykens, E.; Stappaerts, I.; Rutsaert, R. (Aug 2011). "Classic biphasic pulmonary blastoma: a case report and review of the literature.". Lung Cancer 73 (2): 127-32. doi:10.1016/j.lungcan.2011.03.018. PMID 21513998.
  7. ↑ Zhang, GQ.; Fang, XZ.; Wang, BQ.; Sun, W.; Salai, AD. (Apr 2007). "[Clinicopathological and immunohistochemical features of pulmonary blastoma: analysis of 4 cases and review of the literature].". Zhonghua Yi Xue Za Zhi 87 (15): 1040-2. PMID 17672968.
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