Pleuropulmonary blastoma

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Pleuropulmonary blastoma is a very rare primary lung tumour. Traditionally, it was lumped together with well-differentiated fetal adenocarcinoma and classic biphasic pulmonary blastoma, in the category pulmonary blastoma.[1] It is now regarded a separate entity.[2]

General

  • Extremely rare.
  • Tumour of infancy and childhood.[3]
  • May be confused radiologically with CCAM.[4]

Subclassified:[5]

  • Type 1.
  • Type 2.
  • Type 3.

Microscopic

Features:

DDx:

Images

www:

References

  1. 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.
  2. 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.
  3. Ekmekcı, S.; Aysal, A.; Olgun, N.; Olguner, M.; Cakmakci, H.; Ozer, E. (Sep 2013). "Pleuropulmonary Blastoma: A Case Report.". Turk Patoloji Derg. doi:10.5146/tjpath.2013.01191. PMID 24101356.
  4. Lezmi, G.; Verkarre, V.; Khen-Dunlop, N.; Vibhushan, S.; Hadchouel, A.; Rambaud, C.; Copin, MC.; Rittie, JL. et al. (Sep 2013). "FGF10 Signaling differences between type I pleuropulmonary blastoma and congenital cystic adenomatoid malformation.". Orphanet J Rare Dis 8 (1): 130. doi:10.1186/1750-1172-8-130. PMID 24004862.
  5. Bisogno, G.; Brennan, B.; Orbach, D.; Stachowicz-Stencel, T.; Cecchetto, G.; Indolfi, P.; Bien, E.; Ferrari, A. et al. (Sep 2013). "Treatment and prognostic factors in pleuropulmonary blastoma: An EXPeRT report.". Eur J Cancer. doi:10.1016/j.ejca.2013.08.015. PMID 24041875.