Pulmonary blastoma
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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
- Pulmonary blastoma (nih.gov).
- Pulmonary blastoma (nih.gov).
- Fetal type adenocarcinoma (upmc.edu).
- Pulmonary blastoma (surgicalpathologyatlas.com).
- Pulmonary blastoma (flickr.com/Yale Rosen).
IHC
Features:[7]
- TTF-1 +ve.
- S-100 -ve.
- Vimentin -ve.[citation needed]
- 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.
- ↑ 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.
- ↑ 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.