Type I regressed pleuropulmonary blastoma in a 10-year-old boy

  • Hebah Sameer Al AbsiEmail author
  • Sofia Konstantinopoulou
  • Adel Adel Al Junaibi
  • Mohammad Fahed Abdullah
  • Vasudev Sharma
  • Saeeda Al Marzooqi
Case Report


Pleuropulmonary blastoma (PPB) is a rare, malignant tumor of the lung and is the most common primary pulmonary malignancy in children. Here, we report a case of a boy who was diagnosed with type I regressed PPB after being mislabeled with congenital pulmonary malformation. A 10-year-old boy presented to our hospital with a history of worsening dyspnea. Since birth, his clinical status and radiographic images were concerning for congenital lobar emphysema that was managed conservatively. A chest computed tomography (CT) scan confirmed the persistence of a large cystic lesion and a diagnostic and therapeutic cystectomy was performed. Microscopic examination confirmed the presence of PPB type Ir. Patient was managed surgically alone with no added chemotherapy, as there was no overall survival benefit. PPB Ir has an overall favorable clinical outcome. Limited follow-up data are available due to the rarity of the lesion and the overlap with other congenital cystic lung malformations.


Congenital lung malformations Pleuropulmonary blastoma Tumor 



We are grateful for the parents for allowing us to report the case. The authors would like to thank doctor Mohamed Issa Tawil for providing us with the radiology images and doctor Saja Fetyan for her help during the writing process.

Compliance with ethical standards


The authors state no conflict of interest.

Conflict of interest

We wish to confirm that there are no known conflicts of interest associated with this publication and there has been no significant financial support for this work that could have influenced its outcome.

Ethical clearance/informed consent

We asked permission/consent with the patient’s parents that their son will be included in a case report.

Statement of human and animal rights

This case included no experiments on human or animal subjects.


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Copyright information

© Indian Association of Cardiovascular-Thoracic Surgeons 2019

Authors and Affiliations

  1. 1.Division of General Pediatrics, Department of PediatricsSheikh Khalifa Medical CityAbu DhabiUnited Arab Emirates
  2. 2.Department of Pediatrics, Division of PulmonologySheikh Khalifa Medical CityAbu DhabiUnited Arab Emirates
  3. 3.Department of Surgery, Division of Pediatric SurgerySheikh Khalifa Medical CityAbu DhabiUnited Arab Emirates
  4. 4.Department of Pediatrics, Division of HematologySheikh Khalifa Medical CityAbu DhabiUnited Arab Emirates
  5. 5.Department of PathologySheikh Khalifa Medical CityAbu DhabiUnited Arab Emirates
  6. 6.Department of Pathology, College of Medicine and Health SciencesUnited Arab Emirates UniversityAl AinUnited Arab Emirates

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