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]
- DICER1 mutation - common, may be syndromic.[5]
Subclassified:[6]
- Type 1.
- Type 2.
- Type 3.
Microscopic
Features:
DDx:
Images
www:
See also
References
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Pierce, JM.; LaCroix, P.; Heym, K.; Bowman, WP.; Margraf, L.; Iglesias, J.; Ray, A. (Nov 2017). "Pleuropulmonary Blastoma: A Single-center Case Series of 6 Patients.". J Pediatr Hematol Oncol 39 (8): e419-e422. doi:10.1097/MPH.0000000000000972. PMID 28991133.
- ↑ 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.