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Pleuropulmonary blastoma type 3

MedGen UID:
328132
•Concept ID:
C1710499
•
Neoplastic Process
Synonyms: Pleuro-pulmonary blastoma type III; pleuropulmonary blastoma type 3; Pleuropulmonary blastoma type III; Type III Pleuropulmonary Blastoma; type III pleuropulmonary blastoma
SNOMED CT: Pleuro-pulmonary blastoma type III (707673006); Pleuropulmonary blastoma type III (707673006)
 
Monarch Initiative: MONDO:0020557
Orphanet: ORPHA99935

Definition

A pleuropulmonary blastoma characterized by a solid pattern and sarcomatous features. It usually follows an aggressive clinical course. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPleuropulmonary blastoma type 3

Professional guidelines

PubMed

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
Multimodality treatment including whole pleura radiation therapy for DICER1-associated pediatric pleuropulmonary blastoma.
Hui C, Shin DH, Wakeling A, Donaldson SS, Hazard FK, Rangaswami A, Hiniker SM
Pediatr Blood Cancer 2021 Jun;68(6):e29004. Epub 2021 Mar 10 doi: 10.1002/pbc.29004. PMID: 33751747
Treatment and prognostic factors in pleuropulmonary blastoma: an EXPeRT report.
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

Diagnosis

Pleuropulmonary blastoma: a case report.
Ekmekci S, Aysal A, Olgun N, Olguner M, Çakmakci H, Özer E
Turk Patoloji Derg 2015;31(1):68-71. PMID: 25560611

Prognosis

Pleuropulmonary blastoma: a case report.
Ekmekci S, Aysal A, Olgun N, Olguner M, Çakmakci H, Özer E
Turk Patoloji Derg 2015;31(1):68-71. PMID: 25560611

Clinical prediction guides

Pleuropulmonary blastoma: a case report.
Ekmekci S, Aysal A, Olgun N, Olguner M, Çakmakci H, Özer E
Turk Patoloji Derg 2015;31(1):68-71. PMID: 25560611

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