Difference between revisions of "Pulmonary blastoma"
Jump to navigation
Jump to search
(tweak) |
(more) |
||
| Line 1: | Line 1: | ||
'''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> | ||
==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.<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> | ||
**Adults usu. in 40s. | **Adults usu. in 40s. | ||
===Classification=== | ===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 | *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. | ||
Revision as of 03:44, 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.[3]
- Adults usu. in 40s.
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
- ↑ 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.
- ↑ 2.0 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/.
- ↑ Jethava, A.; Dasanu, CA. (Jan 2013). "Adult biphasic pulmonary blastoma.". Conn Med 77 (1): 19-22. PMID 23427368.
- ↑ 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.
- ↑ 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.
- ↑ 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.