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

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVPleuropulmonary blastoma type 2

Pathological process
- Disease
  - Neoplasm
    - Embryonal neoplasm
      - Blastoma
        Pleuropulmonary blastoma
        Pleuropulmonary blastoma type 2

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

Diagnosis and treatment of pleuropulmonary blastoma in children: A single-center report of 41 cases.

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

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

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