CTICasesNeurofibromatosis Type 1 (NF1) with Intrathoracic Neurofibroma and Lung Cysts
Imaging findings
High-resolution CT shows multiple subtle, thin-walled pulmonary cysts scattered throughout the lungs. There are multiple small, cutaneous neurofibromas along the chest wall. A large, homogeneous, well-circumscribed paraspinal and anterior mediastinal mass is seen running along the expected course of the phrenic nerve. Another small, well-defined neurofibroma is noted in the intercostal space.
Key takeaways
Intrathoracic manifestations of neurofibromatosis type 1 (NF1) include multiple neural sheath tumors (such as plexiform or focal neurofibromas arising from the vagus, phrenic, or intercostal nerves) and diffuse lung disease characterized by thin-walled cysts and emphysema-like changes. These benign pulmonary cysts are a recognized but uncommon manifestation of NF1 and must be distinguished from other cystic lung diseases.
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