Pulmonary blastoma

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Pulmonary blastoma is a very rare tumour of the lung.

General

  • Very rare ~ 100 cases in the literature.[1]
  • Abysmal prognosis - 5-year survival ~ 15%.[1]
  • Typical patient <20 years old.[2]
    • Adults usu. in 40s.

Classification

Subtypes:[3]

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

Gross

  • Lung tumour.

Microscopic

Features:

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

DDx:

Images

IHC

Features:[4]

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

See also

References

  1. 1.0 1.1 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/.
  2. Jethava, A.; Dasanu, CA. (Jan 2013). "Adult biphasic pulmonary blastoma.". Conn Med 77 (1): 19-22. PMID 23427368.
  3. 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.
  4. 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.