Type I regressed pleuropulmonary blastoma in a 10-year-old boy
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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.
KeywordsCongenital 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.
- 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
- 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.Dehner LP. Pleuropulmonary blastoma is THE pulmonary blastoma of childhood. Semin Diagn Pathol. 1994;11:144–51.Google Scholar
- 9.PPB R. PPB TYPES: I, II & III. 2018 [cited 2018; Available from: https://www.ppbregistry.org/.