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

• Main Authors: 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
• Format: Article
• Language: English
• Published: Elsevier 2021-09-01
• Series: Radiology Case Reports
• Subjects: pediatrics; integrated imaging; pleuropulmonary blastoma
• Online Access: http://www.sciencedirect.com/science/article/pii/S1930043321004003

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.