Primary pleuropulmonary synovial sarcoma (PPSS) is a rare mesenchymal neoplasm, accounting for less than 0.5% of all primary lung tumours. Presentation is often vague and may include cough, chest pain or dyspnoea. Due to the tumour's rarity, diagnosis can be challenging, and not much is known about the disease process or optimal treatment course. In this case report, we describe an older female patient who underwent blebectomy for recurrent pneumothorax. No masses or suspected lesions were found on CT imaging besides the bleb. The bleb was then found to be PPSS via RT-PCR cytology. This case aims to increase awareness of malignant tumours clinically presenting as recurrent pneumothorax with no discrete lung mass on CT imaging. We also highlight the importance of cytogenetics to confirm the diagnosis of this rare tumour.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10163521 | PMC |
http://dx.doi.org/10.1136/bcr-2023-254560 | DOI Listing |
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.
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.
Cureus
October 2024
Pediatric Surgery, Hamad Medical Corporation, Doha, QAT.
Pleuropulmonary blastoma (PPB) is an extremely rare and highly malignant intrathoracic tumor in children, representing a unique form of aggressive primary lung carcinoma with a strong tendency for local recurrence. In this case report, we present a two-year-old girl who has had recurrent respiratory infections since birth. A chest X-ray revealed an abnormality, prompting a referral to a surgical team, where the lesion was identified as type II PPB based on histological analysis.
View Article and Find Full Text PDFTransl Pediatr
October 2024
Department of Pediatric Surgery, Fujian Children's Hospital (Fujian Branch of Shanghai Children's Medical Center), College of Clinical Medicine for Obstetrics & Gynecology and Pediatrics, Fujian Medical University, Fuzhou, China.
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