“ The chest may be involved in neurofibromatosis type 1 ( Vm Recklinghausen disease), either from n eurofibromata, meningocele or parenchyma...
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The chest may be involved in neurofibromatosis type 1 (Vm Recklinghausen disease), either from neurofibromata, meningocele or parenchymal lung disease.
PLAIN FILM
• Large posterior mediastinal mass (meningocele or neurofibroma).
• Ribbon ribs/posterior scalloping of the vertebral bodies (due to adjacent neurofibroma).
• Look for lung nodules—these may actually be cutaneous neurofibromata.
• Fibrosis at the lung bases.
HRCT/CT
• Progressive, symmetrical basal fibrosis and upper lobe bullae.
• Look for paravertebral masses extending into the spinal canal—soft tissue/some fat density suggests neurofibroma, fluid density suggests a lateral meningocele.
Neurofibromatosis. Chest x-ray demonstrating a large posterior mediastinal mass with splaying and thinning of the ribs.
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