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Pleuropulmonary blastoma type 1

MedGen UID:
318288
Concept ID:
C1710501
Neoplastic Process
Synonyms: Pleuro-pulmonary blastoma type I; pleuropulmonary blastoma type 1; Pleuropulmonary blastoma type I; Type I Pleuropulmonary Blastoma; type I pleuropulmonary blastoma
SNOMED CT: Pleuro-pulmonary blastoma type I (707671008); Pleuropulmonary blastoma type I (707671008)
 
Monarch Initiative: MONDO:0020555
Orphanet: ORPHA99933

Definition

A pleuropulmonary blastoma composed of malignant small cells. Sarcomatous features are absent. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPleuropulmonary blastoma type 1

Professional guidelines

PubMed

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
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
Christosova IR, Avramova BE, Drebov RS, Shivachev HI, Kamenova MA, Bobev DG, Brankov OG
Pediatr Pulmonol 2015 Jul;50(7):698-703. Epub 2014 Apr 1 doi: 10.1002/ppul.23047. PMID: 24692196

Recent clinical studies

Etiology

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
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
Sparber-Sauer M, Tagarelli A, Seitz G, Sorg B, Bien E, Bel-Ami T, Pourtsidis A, Lopez Almaraz R, Koscielniak E, Ferrari A, Orbach D, Bisogno G; EXPeRT and CWS Study Groups
Pediatr Blood Cancer 2021 Dec;68(12):e29268. Epub 2021 Sep 5 doi: 10.1002/pbc.29268. PMID: 34486213
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
Christosova IR, Avramova BE, Drebov RS, Shivachev HI, Kamenova MA, Bobev DG, Brankov OG
Pediatr Pulmonol 2015 Jul;50(7):698-703. Epub 2014 Apr 1 doi: 10.1002/ppul.23047. PMID: 24692196

Diagnosis

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
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
Sparber-Sauer M, Tagarelli A, Seitz G, Sorg B, Bien E, Bel-Ami T, Pourtsidis A, Lopez Almaraz R, Koscielniak E, Ferrari A, Orbach D, Bisogno G; EXPeRT and CWS Study Groups
Pediatr Blood Cancer 2021 Dec;68(12):e29268. Epub 2021 Sep 5 doi: 10.1002/pbc.29268. PMID: 34486213
Christosova IR, Avramova BE, Drebov RS, Shivachev HI, Kamenova MA, Bobev DG, Brankov OG
Pediatr Pulmonol 2015 Jul;50(7):698-703. Epub 2014 Apr 1 doi: 10.1002/ppul.23047. PMID: 24692196
Priest JR, Magnuson J, Williams GM, Abromowitch M, Byrd R, Sprinz P, Finkelstein M, Moertel CL, Hill DA
Pediatr Blood Cancer 2007 Sep;49(3):266-73. doi: 10.1002/pbc.20937. PMID: 16807914

Therapy

Schultz KAP, Harris AK, Nelson AT, Watson D, Lucas JT Jr, Miniati D, Stewart DR, Hagedorn KN, Mize W, Kamihara J, Mitchell SG, Wilson DB, Gettinger K, Rangaswami AA, Harney LA, Rodriguez Galindo C, Bisogno G, Dehner LP, Hill DA, Messinger YH
J Clin Oncol 2023 Feb 1;41(4):778-789. Epub 2022 Sep 22 doi: 10.1200/JCO.21.02925. PMID: 36137255Free PMC Article
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
Grigoletto V, Tagarelli A, Atzeni C, Cecchetto G, Indolfi P, De Pasquale MD, De Leonardis F, Coppadoro B, Sorbara S, Chiaravalli S, Ferrari A, Bisogno G
Tumori 2020 Apr;106(2):126-132. Epub 2019 Sep 5 doi: 10.1177/0300891619871344. PMID: 32270754
Christosova IR, Avramova BE, Drebov RS, Shivachev HI, Kamenova MA, Bobev DG, Brankov OG
Pediatr Pulmonol 2015 Jul;50(7):698-703. Epub 2014 Apr 1 doi: 10.1002/ppul.23047. PMID: 24692196
Bisogno G, Brennan B, Orbach D, Stachowicz-Stencel T, Cecchetto G, Indolfi P, Bien E, Ferrari A, Dommange-Romero F
Eur J Cancer 2014 Jan;50(1):178-84. Epub 2013 Sep 13 doi: 10.1016/j.ejca.2013.08.015. PMID: 24041875

Prognosis

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
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
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
Christosova IR, Avramova BE, Drebov RS, Shivachev HI, Kamenova MA, Bobev DG, Brankov OG
Pediatr Pulmonol 2015 Jul;50(7):698-703. Epub 2014 Apr 1 doi: 10.1002/ppul.23047. PMID: 24692196
Priest JR, Magnuson J, Williams GM, Abromowitch M, Byrd R, Sprinz P, Finkelstein M, Moertel CL, Hill DA
Pediatr Blood Cancer 2007 Sep;49(3):266-73. doi: 10.1002/pbc.20937. PMID: 16807914

Clinical prediction guides

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
Alipour Z, Schultz KAP, Chen L, Harris AK, Gonzalez IA, Pfeifer J, Hill DA, He M, Dehner LP
Pediatr Dev Pathol 2021 Nov-Dec;24(6):523-530. Epub 2021 Jul 15 doi: 10.1177/10935266211027417. PMID: 34266329Free PMC Article
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
Lee M, Kim TI, Jang SJ, Cho KJ, Lee SM, Kim HR, Song JS
Pathobiology 2021;88(3):251-260. Epub 2021 Feb 10 doi: 10.1159/000512957. PMID: 33567437
Vokuhl C, de Leon-Escapini L, Leuschner I
Pediatr Dev Pathol 2017 Nov-Dec;20(6):475-481. Epub 2017 Apr 6 doi: 10.1177/1093526617700945. PMID: 28382840

Recent systematic reviews

Casagrande A, Pederiva F
J Thorac Oncol 2016 Nov;11(11):1837-1845. Epub 2016 Jul 15 doi: 10.1016/j.jtho.2016.06.023. PMID: 27423390

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