Sclerosing Pneumocytoma of the Lungs Arising in a Child With PTEN Mutation. (December 2019)

• Record Type: Journal Article
• Authors: Nasr, Youssef; Bettoli, Marcos; El Demellawy, Dina; Sekhon, Harman; de Nanassy, Joseph
• Is Part Of: Pediatric and developmental pathology. Volume 22:Number 6(2019)
• Page Start: 579
• Page End: 583
• Publication Date: 2019-12
• Subjects: lung -- microscopy -- molecular biology -- molecular oncology -- pediatric -- PediPath -- sclerosing hemangioma -- tumors; Pediatric pathology -- Periodicals; Children -- Diseases -- Periodicals; Diagnosis, Laboratory -- Periodicals; Abnormalities, Human -- Periodicals; Child development -- Periodicals; Pediatrics -- Periodicals; 616.07
• DOI: 10.1177/1093526619851423
• Languages: English
• ISSNs: 1093-5266

Abstract

We report a rare case of sclerosing pneumocytoma occurring in a child with PTEN mutation. A 13-year-old female presented to the emergency department of an adult hospital following 2 to 3 days of upper respiratory tract infection symptoms. A primary lung lesion was discovered during her initial chest X-ray to rule out pneumonia. The patient underwent an uneventful thoracoscopic right upper lobe segmentectomy. The pathology demonstrated a sclerosing pneumocytoma of the lung. She tested positive for PTEN hamartoma tumor syndrome with a pathogenic variant at c.388 C > T. The PTEN mutation was also identified in the sclerosing pneumocytoma. Further study of PTEN mutation in sclerosing pneumocytoma is warranted.