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Unusual thoracic tumor in a teenager: A rare occurrence

Pleuropulmonary blastoma (PPB) is a rare malignant lung tumor in the pediatric population and occurs mainly in young children. Its clinical presentation is usually nonspecific. We report a rare occurrence of this tumor in a 15-year-old girl, who presented with symptoms mimicking respiratory tract in...

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Detalles Bibliográficos
Autores principales: Muhamad, Tasnim, Amir Sayuti, Khairil, Hasnita Ismail Mokhtar, Noor, Hayati Yunus, Nor, Syafawati Ismail, Noor
Formato: Online Artículo Texto
Lenguaje:English
Publicado: HBKU Press 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8894663/
https://www.ncbi.nlm.nih.gov/pubmed/35291285
http://dx.doi.org/10.5339/qmj.2022.12
Descripción
Sumario:Pleuropulmonary blastoma (PPB) is a rare malignant lung tumor in the pediatric population and occurs mainly in young children. Its clinical presentation is usually nonspecific. We report a rare occurrence of this tumor in a 15-year-old girl, who presented with symptoms mimicking respiratory tract infection and was nonresponsive to the initial treatment. Imaging investigations revealed a large solid lesion in the left hemithorax with a mass effect on the adjacent structures. Biopsy demonstrated primitive cells with blastematous appearances, and the stroma cells were positive for vimentin and desmin, consistent with PPB. Unfortunately, she died from neutropenic sepsis while undergoing chemotherapy. This report highlights the epidemiology of PPB, its imaging and histopathological features, overview of prognosis, and clinical management.