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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

Multimodality Imaging of Pleuropulmonary Blastoma: Pearls, Pitfalls, and Differential Diagnosis.
Leung SS, Donuru A, Kandula V, Parekh MR, Saul D
Semin Ultrasound CT MR 2022 Feb;43(1):61-72. Epub 2021 May 15 doi: 10.1053/j.sult.2021.05.007. PMID: 35164911
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
Pulmonary Sarcomatoid carcinoma: a surgical diagnosis and prognostic factors.
Smadhi H, Boudaya MS, Abdannadher M, BenAbdelghaffar H, Kamoun H, Ayadi A, Fekih L, Marghli A, Megdiche ML
Tunis Med 2019 Jan;97(1):128-132. PMID: 31535704
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
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

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
Multimodality Imaging of Pleuropulmonary Blastoma: Pearls, Pitfalls, and Differential Diagnosis.
Leung SS, Donuru A, Kandula V, Parekh MR, Saul D
Semin Ultrasound CT MR 2022 Feb;43(1):61-72. Epub 2021 May 15 doi: 10.1053/j.sult.2021.05.007. PMID: 35164911
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
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

Therapy

Pulmonary blastoma is successfully treated with immunotherapy and targeted therapy.
Chen Y, Chen H, Yu R, Zeng X, Tian D, Pu Q, Liu Y
Lung Cancer 2024 Mar;189:107476. Epub 2024 Jan 24 doi: 10.1016/j.lungcan.2024.107476. PMID: 38280290
Treatment and prognosis of pleuropulmonary blastoma: A single-center report of 31 cases.
Wang F, Zhuang L, Liang J, Li L, Wang H, Tang J, Zeng J, Liu W
Pediatr Pulmonol 2022 Sep;57(9):2237-2243. Epub 2022 Jun 13 doi: 10.1002/ppul.25930. PMID: 35510654
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
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

Prognosis

Treatment and prognosis of pleuropulmonary blastoma: A single-center report of 31 cases.
Wang F, Zhuang L, Liang J, Li L, Wang H, Tang J, Zeng J, Liu W
Pediatr Pulmonol 2022 Sep;57(9):2237-2243. Epub 2022 Jun 13 doi: 10.1002/ppul.25930. PMID: 35510654
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
Pulmonary Sarcomatoid carcinoma: a surgical diagnosis and prognostic factors.
Smadhi H, Boudaya MS, Abdannadher M, BenAbdelghaffar H, Kamoun H, Ayadi A, Fekih L, Marghli A, Megdiche ML
Tunis Med 2019 Jan;97(1):128-132. PMID: 31535704
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

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
Type I and Ir pleuropulmonary blastoma (PPB): A report from the International PPB/DICER1 Registry.
Nelson AT, Harris AK, Watson D, Miniati D, Finch M, Kamihara J, Mitchell SG, Wilson DB, Gettinger K, Rangaswami AA, Campos JM, Lederman S, Feltis BA, Vasta LM, Harney LA, Stewart DR, Dehner LP, Messinger YH, Hill DA, Schultz KAP
Cancer 2023 Feb 15;129(4):600-613. Epub 2022 Dec 20 doi: 10.1002/cncr.34593. PMID: 36541021Free PMC Article
Epidemiological features and survival outcomes in patients with malignant pulmonary blastoma: a US population-based analysis.
Bu X, Liu J, Wei L, Wang X, Chen M
BMC Cancer 2020 Aug 26;20(1):811. doi: 10.1186/s12885-020-07323-0. PMID: 32847556Free PMC Article
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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