Type II pleuropulmonary blastoma in a 3-years-old female with dyspnea: a case report and review of literature

Autor: Dolores Ferrara, MD, Francesco Esposito, MD, Eugenio Rossi, MD, Parvin Gholami Shangolabad, MD, Vittoria D'Onofrio, MD, Delfina Bifano, MD, Diana Baldari, MD, Carmela Brillantino, MD, Raffaele Zeccolini, MD, Massimo Zeccolini, MD
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Zdroj: Radiology Case Reports, Vol 16, Iss 9, Pp 2736-2741 (2021)
Druh dokumentu: article
ISSN: 1930-0433
DOI: 10.1016/j.radcr.2021.06.022
Popis: Pleuropulmonary blastoma (PPB) is a rare but aggressive pediatric tumor originates from either lung or pleura. It was recently linked to the DICER I mutation as a part of predisposition syndrome for different type of tumor. It is characterized histologically by a primitive, variably mixed blastomatous and sarcomatous tissue. PPB is classified into four subtypes: cystic (type I and type Ir); cystic and solid (type II); solid (type III).PPB has no characteristic imaging findings. Integrated imaging can help to make a differential diagnosis and to recognize the subtypes in order to set up therapy. An early recognition and differentiation from congenital airway malformations and other benign cysts are very important.The treatment consists in a multimodal therapy including surgery and chemoterapy.We report a case of 3 years old female admitted at our hospital with fever, non productive cough and dyspnea, who was diagnosed with type II PPB.
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