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Pleuropulmonary blastoma type 2

MedGen UID:
328133
Concept ID:
C1710500
Neoplastic Process
Synonyms: Pleuro-pulmonary blastoma type II; pleuropulmonary blastoma type 2; Pleuropulmonary blastoma type II; Type II Pleuropulmonary Blastoma; type II pleuropulmonary blastoma
SNOMED CT: Pleuro-pulmonary blastoma type II (707672001); Pleuropulmonary blastoma type II (707672001)
 
Monarch Initiative: MONDO:0020556
Orphanet: ORPHA99934

Definition

A pleuropulmonary blastoma composed of malignant small cells and characterized by the presence of a sarcomatous component. It usually follows an aggressive clinical course. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPleuropulmonary blastoma type 2

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
Zhang N, Zeng Q, Ma X, Chen C, Yu J, Zhang X, Yan D, Xu C, Liu D, Zhang Q
J Pediatr Surg 2020 Jul;55(7):1351-1355. Epub 2019 Jun 27 doi: 10.1016/j.jpedsurg.2019.06.009. PMID: 31277979
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

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
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
Zhang N, Zeng Q, Ma X, Chen C, Yu J, Zhang X, Yan D, Xu C, Liu D, Zhang Q
J Pediatr Surg 2020 Jul;55(7):1351-1355. Epub 2019 Jun 27 doi: 10.1016/j.jpedsurg.2019.06.009. PMID: 31277979
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

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
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
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
Zhang N, Zeng Q, Ma X, Chen C, Yu J, Zhang X, Yan D, Xu C, Liu D, Zhang Q
J Pediatr Surg 2020 Jul;55(7):1351-1355. Epub 2019 Jun 27 doi: 10.1016/j.jpedsurg.2019.06.009. PMID: 31277979
Anand R, Narula MK, Chadha R, Chander J, Jain M
J Indian Med Assoc 2013 Feb;111(2):128-9. PMID: 24003574

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
Geller JI, Pressey JG, Smith MA, Kudgus RA, Cajaiba M, Reid JM, Hall D, Barkauskas DA, Voss SD, Cho SY, Berg SL, Dome JS, Fox E, Weigel BJ
Cancer 2020 Dec 15;126(24):5303-5310. Epub 2020 Sep 11 doi: 10.1002/cncr.33195. PMID: 32914879Free PMC Article
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
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

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
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
Zhang N, Zeng Q, Ma X, Chen C, Yu J, Zhang X, Yan D, Xu C, Liu D, Zhang Q
J Pediatr Surg 2020 Jul;55(7):1351-1355. Epub 2019 Jun 27 doi: 10.1016/j.jpedsurg.2019.06.009. PMID: 31277979
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

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
Messinger YH, Stewart DR, Priest JR, Williams GM, Harris AK, Schultz KA, Yang J, Doros L, Rosenberg PS, Hill DA, Dehner LP
Cancer 2015 Jan 15;121(2):276-85. Epub 2014 Sep 10 doi: 10.1002/cncr.29032. PMID: 25209242Free PMC Article
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

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