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

Autor: Al Absi HS; Division of General Pediatrics, Department of Pediatrics, Sheikh Khalifa Medical City, Al Karama street, Abu Dhabi, United Arab Emirates., Konstantinopoulou S; Department of Pediatrics, Division of Pulmonology, Sheikh Khalifa Medical City, Abu Dhabi, United Arab Emirates., Al Junaibi AA; Department of Surgery, Division of Pediatric Surgery, Sheikh Khalifa Medical City, Abu Dhabi, United Arab Emirates., Abdullah MF; Department of Pediatrics, Division of Hematology, Sheikh Khalifa Medical City, Abu Dhabi, United Arab Emirates., Sharma V; Department of Pathology, Sheikh Khalifa Medical City, Abu Dhabi, United Arab Emirates., Al Marzooqi S; Department of Pathology, College of Medicine and Health Sciences, United Arab Emirates University, Al Ain, United Arab Emirates.
Jazyk: angličtina
Zdroj: Indian journal of thoracic and cardiovascular surgery [Indian J Thorac Cardiovasc Surg] 2019 Oct; Vol. 35 (4), pp. 579-583. Date of Electronic Publication: 2019 May 08.
DOI: 10.1007/s12055-019-00814-1
Abstrakt: 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.
Competing Interests: DisclosureThe authors state no conflict of interest.Conflict of interestWe 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.
(© Indian Association of Cardiovascular-Thoracic Surgeons 2019.)
Databáze: MEDLINE
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