Congenital Pulmonary Myofibroblastic Tumor

  • Megan K. Dishop


Congenital pulmonary myofibroblastic tumor (CPMT) is a very rare benign lung tumor occurring in fetuses and neonates. It typically results in intrauterine fetal demise, hydrops fetalis, or severe respiratory distress at birth. Approximately 10 cases of CPMT have been described in the literature, including cases reported under names such as “bronchopulmonary leiomyosarcoma,” “bronchopulmonary fibrosarcoma,” and “congenital fibroleiomyosarcoma.” The tumor is thought to arise from the pluripotent peribronchial mesenchyme, a precursor to the myofibroblastic phenotype. On chest radiograph, a mass lung lesion causing flattening of the diaphragm and medistinal shift may be seen.


Chest Radiograph Spindle Cell Cytogenetic Analysis Lung Lesion Distinctive Tumor 
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Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • Megan K. Dishop
    • 1
  1. 1.Texas Children’s HospitalHoustonUSA

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