CTICasesNeurofibromatosis Type 1 with Extensive Mediastinal Neurofibromas
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Imaging findings
Chest radiograph shows mediastinal widening. CT reveals lobulated, low-attenuation, minimally enhancing masses scattered throughout the mediastinum, causing tracheal compression. These masses appear to arise along nerves, possibly the phrenic nerves. Skin lesions (neurofibromas) are visible. Abdominal imaging shows numerous neurofibromas in the psoas and other muscles, indicating extensive disease.
Key takeaways
Neurofibromatosis Type 1 can present with extensive neurofibromas, including intrathoracic involvement, leading to mediastinal masses and tracheal compression. While surgery can relieve compression, the widespread nature of the disease highlights the systemic manifestations of NF1.
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