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Pulmonary blastoma

MedGen UID:
61645
•Concept ID:
C0206629
•
Neoplastic Process
Synonyms: Blastoma, Pulmonary; Blastomas, Pulmonary; Pulmonary Blastoma; Pulmonary Blastomas
SNOMED CT: Pulmonary blastoma (189815007); Pneumoblastoma (189815007); Pulmonary blastoma (43149009); Pneumoblastoma (43149009)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Non-Mendelian inheritance (Orphanet)
 
Monarch Initiative: MONDO:0005933
Orphanet: ORPHA64741

Definition

A biphasic primary lung neoplasm, belonging to the group of sarcomatoid lung carcinomas (SLCs). The tumor contains both an epithelial well-differentiated component, showing tubular architecture resembling the normal fetal lung, and a mesenchymal undifferentiated stroma with a so-called ''blastema-like'' configuration that resembles an embryonic lung. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPulmonary blastoma
Follow this link to review classifications for Pulmonary blastoma in Orphanet.

Professional guidelines

PubMed

SMARCA4 as a support for the differential diagnosis of poorly differentiated lung carcinomas.
Panozzi M, Alì G, Proietti A, Melfi F, Zirafa CC, Lucchi M, Fontanini G
Pathologica 2023 Jun;115(3):164-171. doi: 10.32074/1591-951X-847. PMID: 37387441Free PMC Article
Pulmonary blastoma: a review with special emphasis on prognosis and treatment.
Larsen H, Sørensen JB
Cancer Treat Rev 1996 May;22(3):145-60. doi: 10.1016/s0305-7372(96)90000-6. PMID: 8841388
Primary sarcomas of the lung.
Suster S
Semin Diagn Pathol 1995 May;12(2):140-57. PMID: 7638447

Recent clinical studies

Etiology

Pulmonary blastoma: a comprehensive overview of a rare entity.
Tsamis I, Chachali SP, Gomatou G, Trontzas I, Mitsogianni M, Syrigos N, Vamvakaris I, Kotteas E
Adv Respir Med 2021;89(5):511-519. doi: 10.5603/ARM.a2021.0085. PMID: 34725809
Congenital pulmonary airway malformations: state-of-the-art review for pediatrician's use.
Leblanc C, Baron M, Desselas E, Phan MH, Rybak A, Thouvenin G, Lauby C, Irtan S
Eur J Pediatr 2017 Dec;176(12):1559-1571. Epub 2017 Oct 19 doi: 10.1007/s00431-017-3032-7. PMID: 29046943
Phenotype and immunophenotype of the most common pediatric tumors.
Picarsic J, Reyes-Múgica M
Appl Immunohistochem Mol Morphol 2015 May-Jun;23(5):313-26. doi: 10.1097/PAI.0000000000000068. PMID: 25390357
Congenital lung lesions.
Puligandla PS, Laberge JM
Clin Perinatol 2012 Jun;39(2):331-47. doi: 10.1016/j.clp.2012.04.009. PMID: 22682383
Malignant biphasic tumors of the lungs.
Weissferdt A, Moran CA
Adv Anat Pathol 2011 May;18(3):179-89. doi: 10.1097/PAP.0b013e318216980c. PMID: 21490435

Diagnosis

Congenital Pulmonary Airway Malformations With a Reconsideration and Current Perspective on the Stocker Classification.
Dehner LP, Schultz KAP, Hill DA
Pediatr Dev Pathol 2023 May-Jun;26(3):241-249. Epub 2023 Feb 21 doi: 10.1177/10935266221146823. PMID: 37334833
Congenital pulmonary airway malformations: state-of-the-art review for pediatrician's use.
Leblanc C, Baron M, Desselas E, Phan MH, Rybak A, Thouvenin G, Lauby C, Irtan S
Eur J Pediatr 2017 Dec;176(12):1559-1571. Epub 2017 Oct 19 doi: 10.1007/s00431-017-3032-7. PMID: 29046943
Pleuropulmonary blastoma.
Anand R, Narula MK, Chadha R, Chander J, Jain M
J Indian Med Assoc 2013 Feb;111(2):128-9. PMID: 24003574
Congenital lung lesions.
Puligandla PS, Laberge JM
Clin Perinatol 2012 Jun;39(2):331-47. doi: 10.1016/j.clp.2012.04.009. PMID: 22682383
Malignant biphasic tumors of the lungs.
Weissferdt A, Moran CA
Adv Anat Pathol 2011 May;18(3):179-89. doi: 10.1097/PAP.0b013e318216980c. PMID: 21490435

Therapy

Classic biphasic pulmonary blastoma: A case report and review of the literature.
Le Caer H, Teissier E, Barriere JR, Venissac N
Crit Rev Oncol Hematol 2018 May;125:48-50. Epub 2018 Mar 3 doi: 10.1016/j.critrevonc.2018.02.009. PMID: 29650276
The Radiologic and Pathologic Diagnosis of Biphasic Pulmonary Blastoma.
Nemeh F, Kuo AH, Ross J, Restrepo CS
J Radiol Case Rep 2017 Sep;11(9):10-21. Epub 2017 Sep 30 doi: 10.3941/jrcr.v11i9.3153. PMID: 29299105Free PMC Article
Congenital lung lesions.
Puligandla PS, Laberge JM
Clin Perinatol 2012 Jun;39(2):331-47. doi: 10.1016/j.clp.2012.04.009. PMID: 22682383
Classic biphasic pulmonary blastoma: a case report and review of the literature.
Van Loo S, Boeykens E, Stappaerts I, Rutsaert R
Lung Cancer 2011 Aug;73(2):127-32. Epub 2011 Apr 21 doi: 10.1016/j.lungcan.2011.03.018. PMID: 21513998
Pulmonary blastoma.
Prasad A, Choudhry P, Sarin YK, Saran RK
Indian J Pediatr 2001 Aug;68(8):793-6. doi: 10.1007/BF02752427. PMID: 11563261

Prognosis

DICER1 tumor predisposition syndrome: an evolving story initiated with the pleuropulmonary blastoma.
González IA, Stewart DR, Schultz KAP, Field AP, Hill DA, Dehner LP
Mod Pathol 2022 Jan;35(1):4-22. Epub 2021 Oct 1 doi: 10.1038/s41379-021-00905-8. PMID: 34599283Free PMC Article
Classic biphasic pulmonary blastoma: A case report and review of the literature.
Le Caer H, Teissier E, Barriere JR, Venissac N
Crit Rev Oncol Hematol 2018 May;125:48-50. Epub 2018 Mar 3 doi: 10.1016/j.critrevonc.2018.02.009. PMID: 29650276
Malignant biphasic tumors of the lungs.
Weissferdt A, Moran CA
Adv Anat Pathol 2011 May;18(3):179-89. doi: 10.1097/PAP.0b013e318216980c. PMID: 21490435
Pulmonary blastoma.
Prasad A, Choudhry P, Sarin YK, Saran RK
Indian J Pediatr 2001 Aug;68(8):793-6. doi: 10.1007/BF02752427. PMID: 11563261
Pulmonary blastoma.
Peacock MJ, Whitwell F
Thorax 1976 Apr;31(2):197-204. doi: 10.1136/thx.31.2.197. PMID: 941110Free PMC Article

Clinical prediction guides

Congenital Pulmonary Airway Malformations With a Reconsideration and Current Perspective on the Stocker Classification.
Dehner LP, Schultz KAP, Hill DA
Pediatr Dev Pathol 2023 May-Jun;26(3):241-249. Epub 2023 Feb 21 doi: 10.1177/10935266221146823. PMID: 37334833
Pleuropulmonary Blastoma in Pediatric Lung Lesions.
Kunisaki SM, Lal DR, Saito JM, Fallat ME, St Peter SD, Fox ZD, Heider A, Chan SS, Boyd KP, Burns RC, Deans KJ, Gadepalli SK, Hirschl RB, Kabre R, Landman MP, Leys CM, Mak GZ, Minneci PC, Wright TN, Helmrath MA; MIDWEST PEDIATRIC SURGERY CONSORTIUM
Pediatrics 2021 Apr;147(4) Epub 2021 Mar 24 doi: 10.1542/peds.2020-028357. PMID: 33762310
Type I pleuropulmonary blastoma: pathology and biology study of 51 cases from the international pleuropulmonary blastoma registry.
Hill DA, Jarzembowski JA, Priest JR, Williams G, Schoettler P, Dehner LP
Am J Surg Pathol 2008 Feb;32(2):282-95. doi: 10.1097/PAS.0b013e3181484165. PMID: 18223332
Pulmonary blastomas.
Koss MN, Hochholzer L, O'Leary T
Cancer 1991 May 1;67(9):2368-81. doi: 10.1002/1097-0142(19910501)67:9<2368::aid-cncr2820670926>3.0.co;2-g. PMID: 1849449
Pleuropulmonary blastoma. The so-called pulmonary blastoma of childhood.
Manivel JC, Priest JR, Watterson J, Steiner M, Woods WG, Wick MR, Dehner LP
Cancer 1988 Oct 15;62(8):1516-26. doi: 10.1002/1097-0142(19881015)62:8<1516::aid-cncr2820620812>3.0.co;2-3. PMID: 3048630

Recent systematic reviews

Multimorbidity and Genetic Characteristics of DICER1 Syndrome Based on Systematic Review.
Cai S, Zhao W, Nie X, Abbas A, Fu L, Bihi S, Feng G, Liu T, Lv Y, Ma X, Peng X
J Pediatr Hematol Oncol 2017 Jul;39(5):355-361. doi: 10.1097/MPH.0000000000000715. PMID: 27906793

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