CTICasesPlexiform Neurofibromas in Neurofibromatosis Type 1
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Imaging findings
CT scan of the chest demonstrates bilateral, symmetric, posterior rib notching and scalloping of both lung apices. There are multiple low-attenuation paraspinal and mediastinal masses, including a long, tubular, low-attenuation mass tracking the entire course of the left phrenic nerve, and several intercostal nerve masses.
Key takeaways
Neurofibromatosis type 1 (NF1) is associated with plexiform and discrete neurofibromas arising from intrathoracic nerves, including the vagus, phrenic, and intercostal nerves. Intercostal neurofibromas can cause pressure-induced posterior rib notching, mimicking aortic coarctation, while apical chest wall neurofibromas produce characteristic apical lung scalloping.
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