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Type III pleuropulmonary blastoma: A case report.
Oncol Lett
March 2025
Department of Pathology, Affiliated Hospital of Zunyi Medical University, Zunyi, Guizhou 563000, P.R. China.
Pleuropulmonary blastoma (PPB) is an uncommon malignant neoplasm occurring in infants. The disease is intimately linked to mutations in the Dcr-1 homolog and ribonuclease type III (DICER1) genes. Imaging techniques are crucial for diagnosing PPB, yet distinguishing PPB from other pulmonary masses proves challenging.
View Article and Find Full Text PDFPulmonary Consequences of Surgical Treatment in Children's Primary Lung Tumors: A National Retrospective Study.
Pediatr Blood Cancer
January 2025
Pediatric Hematology and Oncology Department, University Hospital of Caen, Caen, France.
Background And Aims: Primary lung tumors (PLTs) in children are rare, and surgery remains the key to ensure remission. Here we describe the PLTs clinical characteristics, their management, and the pulmonary outcome following surgery.
Methods: We carried out a French national cohort of pediatric PLTs from 2013 to 2023 from the FRACTURE rare pediatric tumors national database.
Cystic masses of the pediatric lung: update on congenital pulmonary airway malformation and its differential diagnosis.
Virchows Arch
December 2024
Department of Pathology, Boston Children's Hospital & Harvard Medical School, 300 Longwood Avenue, Boston, MA, 02115, USA.
Localized cystic lung lesions in pediatric patients encompass a spectrum of benign and rare malignant conditions that are quite distinct from cystic lung disease arising in adulthood. The majority have historically fallen under the diagnostic category of "congenital pulmonary airway malformation," a term that has been used to denote a diverse group of diseases ranging in etiology from ectopia to bronchial atresia to mosaic oncogenic mutation or neoplasia. This article reviews the clinical characteristics, gross and histologic features, and pathogenetic underpinnings of congenital pulmonary airway malformation as well as lesions that enter its histologic differential diagnosis.
View Article and Find Full Text PDFPleuropulmonary blastoma and DICER1-related tumor predisposition: from clinicopathologic observations to clinical trial.
Curr Opin Pediatr
February 2025
International Pleuropulmonary Blastoma/DICER1 Registry.
Purpose Of Review: Pleuropulmonary blastoma (PPB) is a rare primary lung neoplasm of infancy and childhood. The purpose of this review is to highlight recent developments in our understanding of PPB and research strategies to facilitate future rare cancer research.
Recent Findings: The International PPB/DICER1 Registry has recently assembled the largest-ever cohorts of type I and Ir PPB and type II and III PPB.
Classic biphasic pulmonary blastoma with endobronchial invasion in a 16-year-old male: A rare case report.
Respir Med Case Rep
November 2024
Head of Pathology Department, Bairouni Hospital, Damascus University, Damascus, Syria.
Pulmonary blastoma (PB) is a rare and aggressive lung neoplasm, representing 0.25 %-0.50 % of lung cancers and characterized by biphasic histology with both epithelial and mesenchymal components.
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