Rhabdomyosarcoma arising within congenital pulmonary cysts: report of three cases

Area of Science:

• Pediatric Oncology
• Pulmonary Medicine
• Pathology

Background:

• Primary pulmonary rhabdomyosarcoma is exceptionally rare in children.
• Congenital lung malformations, such as cystic adenomatoid malformations and bronchogenic cysts, are known to have malignant potential.
• Early diagnosis of pediatric lung masses is crucial for improved outcomes.

Purpose of the Study:

• To report three cases of primary pulmonary rhabdomyosarcoma in young children.
• To investigate the association between congenital lung cysts and the development of pulmonary rhabdomyosarcoma.
• To raise awareness of the risk of malignancy in pediatric lung cysts.

Main Methods:

• Case series review of three pediatric patients treated at British Columbia Children's Hospital over 9 months.
• Clinical presentation, radiographic findings (chest x-ray), and pathological origin of the tumors were analyzed.
• Histopathological confirmation of rhabdomyosarcoma arising from congenital lung cysts.

Main Results:

• Three pediatric patients (24-42 months) were diagnosed with primary pulmonary rhabdomyosarcoma.
• Two patients presented with spontaneous pneumothorax and cystic lung changes; one with chronic cough, fever, and failure to thrive.
• All three tumors originated within congenital lung cysts: one bronchogenic cyst and two cystic adenomatoid malformations.

Conclusions:

• There is a significant risk of rhabdomyosarcoma developing within congenital pulmonary malformations.
• Congenital lung cysts should be considered a potential precursor for pediatric pulmonary rhabdomyosarcoma.
• Increased vigilance and further research are warranted for early detection and management.