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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
  • 13 Downloads

Abstract

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.

Keywords

Congenital lung malformations Pleuropulmonary blastoma Tumor 

Notes

Acknowledgments

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

Disclosure

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.

References

  1. 1.
    Hill DA, Jarzembowski JA, Priest JR, Williams G, Schoettler P, Dehner LP. Type I pleuropulmonary blastoma: pathology and biology study of 51 cases from the international pleuropulmonary blastoma registry. Am J Surg Pathol. 2008;32:282–95.CrossRefGoogle Scholar
  2. 2.
    Manivel JC, Priest JR, Watterson J, et al. Pleuropulmonary blastoma. The so-called pulmonary blastoma of childhood. Cancer. 1988;62:1516–26.CrossRefGoogle Scholar
  3. 3.
    Dishop MK, Kuruvilla S. Primary and metastatic lung tumors in the pediatric population: a review and 25-year experience at a large children's hospital. Arch Pathol Lab Med. 2008;132:1079–103.Google Scholar
  4. 4.
    Messinger YH, Stewart DR, Priest JR, et al. Pleuropulmonary blastoma: a report on 350 central pathology-confirmed pleuropulmonary blastoma cases by the international Pleuropulmonary Blastoma registry. Cancer. 2015;121:276–85.CrossRefGoogle Scholar
  5. 5.
    Priest JR, Hill DA, Williams GM, et al. Type I pleuropulmonary blastoma: a report from the international Pleuropulmonary Blastoma registry. J Clin Oncol. 2006;24:4492–8.CrossRefGoogle Scholar
  6. 6.
    Gallardo AM, Alvarez de la Rosa RM, De Luis EJF, Mendoza RL, Padilla PAI, Troyano LJ. Antenatal ultrasound diagnosis and neonatal results of the congenital cystic adenomatoid malformation of the lung. Rev Chil Pediatr. 2018;89:224–30.CrossRefGoogle Scholar
  7. 7.
    Haider F, Al Saad K, Al-Hashimi F, Al-Hashimi H. It's rare so be aware: Pleuropulmonary Blastoma mimicking congenital pulmonary airway malformation. Thorac Cardiovasc Surg Rep. 2017;6:e10–e4.Google Scholar
  8. 8.
    Dehner LP. Pleuropulmonary blastoma is THE pulmonary blastoma of childhood. Semin Diagn Pathol. 1994;11:144–51.Google Scholar
  9. 9.
    PPB R. PPB TYPES: I, II & III. 2018 [cited 2018; Available from: https://www.ppbregistry.org/.
  10. 10.
    Hill DA, Ivanovich J, Priest JR, et al. DICER1 mutations in familial pleuropulmonary blastoma. Science. 2009;325:965.CrossRefGoogle Scholar

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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