Rationale: Pulmonary blastoma is a rare primary lung cancer that can be categorized into adult type and child type. The clinical symptoms and imaging features of pulmonary blastoma are nonspecific, making it difficult to diagnose preoperatively. Postoperative pathology with immunohistochemical staining can help diagnosis.
Patient Concerns: A 53-year-old male had chest tightness and shortness of breath.
Diagnoses: The patient was diagnosed as pleural pulmonary blastoma based on computed tomography (CT) scan, pathology, immunohistochemistry, and molecular pathology. CT examination showed solid mass on the upper lobe of the left lung Intraoperative observation found that tumor tissue was gray with tough texture. The surrounding lung tissue showed AE1/AE3 (+), Vimentin (+), and CD34 (+) staining. No epidermal growth factor receptor gene mutation was detected.
Interventions: The left lobe resection plus mediastinal lymph node dissection were performed. After the operation, patient received paclitaxel combined with nedaplatin chemotherapy for 4 times.
Outcomes: Four months later, left pleural metastasis, and mediastinal lymph node metastasis was found. The patient died 15 months later.
Lessons: Pleural pulmonary blastoma is a malignant tumor with rare pathological features that is easy to relapse and metastasis with poor prognosis. Surgical treatment preferably, lobectomy plus mediastinal lymph node dissection, is the first treatment option. The overall prognosis is poor.
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http://dx.doi.org/10.1097/MD.0000000000008918 | DOI Listing |
Biology (Basel)
January 2025
Division of Thoracic Surgery, Cantonal Hospital Lucerne, 6000 Lucerne, Switzerland.
In 2001, two enzyme-encoding genes were recognized in the fruit fly . The genetic material, labeled and , encodes ribonuclease-type enzymes with slightly diverse target substrates. The human orthologue is .
View Article and Find Full Text PDFOncol 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 PDFPediatr Blood Cancer
March 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.
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 PDFCurr 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.
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