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

ABSTRACT: 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.

SUBMITTER: Ferrara D

PROVIDER: S-EPMC8319522 | biostudies-literature | 2021 Sep

REPOSITORIES: biostudies-literature

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

Ferrara Dolores D Esposito Francesco F Rossi Eugenio E Shangolabad Parvin Gholami PG D'Onofrio Vittoria V Bifano Delfina D Baldari Diana D Brillantino Carmela C Zeccolini Raffaele R Zeccolini Massimo M

Radiology case reports 20210718 9

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 imagi ...[more]

PMID: 34345335

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Project description:Introduction and importanceIn its complex way of embryonic evolvement, the diaphragmatic membrane can be involved with various disorders that may partially or entirely not develop. Agenesis of the diaphragm is the term that refers to this maldevelopment. It is the more severe form of congenital diaphragmatic hernia in which intra-abdominal viscera protrude into the thoracic cavity, causing respiratory and gastrointestinal problems. Most neonates delivered with diaphragmatic agenesis do not live more than hours to days of the severity of lung immaturity. However, less than 20 affected cases have been reported so far that survived to childhood and even their adulthood period treated surgically or conservatively. We have reported a case of neglected left hemi-diaphragmatic agenesis for more than six decades, then reviewed all adult diaphragmatic agenesis cases available in the literature for 74 years.Case presentationA 65-year-old female complaining of worsened dyspnea during the last four months, a chronic history of short breath since her fourth decade of life, and recent surgery with the diagnosis of a diaphragmatic hernia, underwent the thoracotomy twice in which unilateral diaphragmatic hernia was diagnosed then repaired.Clinical discussionFor the recurrence of her symptoms, she underwent a second thoracotomy, in which the final diagnosis of left hemi-diaphragmatic agenesis was made. A dual mesh patch constructed the defect. The post-operation period was uneventful. We only found 17 cases of adult hemi or bilateral agenesis of the diaphragm reviewing the main medical literature such as Medline and Web of Science. The conservative and operative treatment managements were equal for eight patients in each of them. One of them refused therapy, and one was non-declared in the study. As in congenital diaphragmatic hernia, the most typical side was the left in 10 out of 18. The most complaints patients had followed by coughing and bowel obstruction was dyspnea and dyspepsia.ConclusionNear the total of the diaphragmatic agenesis cases die in the neonatal population; remained undiagnosed or during an autopsy found. However, typically rare in the adult population, respiratory and digestive disorders are the most prevalent. It is difficult initially because diagnosing is intraoperatively, and no modality is available to help the examiner physician diagnose perinatally.

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

Publications

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

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