Difference between revisions of "Pulmonary blastoma"

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*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 | URL = http://bjr.birjournals.org/content/78/929/437.full }}</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>


===Classification===
===Classification===

Revision as of 03:49, 16 October 2013

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

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]

Classification

Traditionally subdivided into:[4]

  • Well-differentiated fetal adenocarcinoma (WDFA).
  • Classic biphasic pulmonary blastoma (CBPB).
  • Pleuropulmonary blastoma.
    • Now considered a separate entity.[5]

Gross

  • Lung tumour.

Microscopic

Features:

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

DDx:

Images

IHC

Features:[6]

  • TTF-1 +ve.
  • S-100 -ve.
  • Vimentin -ve.
  • EMA -ve.

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. 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.
  5. 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.
  6. 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.