Pulmonary blastoma

From Libre Pathology
Jump to navigation Jump to search

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. //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.