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Localized primary tracheobronchial AL amyloidosis with posterior wall sparing: A case report
Connor W Smith1, Elias Lugo-Fagundo1,2, Zahra F Rahmatullah1
1The Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
None:
Primary amyloidosis is characterized by extracellular deposition of misfolded immunoglobulin light chains, commonly involving the respiratory tract in its systemic form. Symptomatic, localized manifestations such as primary tracheobronchial amyloidosis are rare and often present with non-specific symptoms including cough, dyspnea, hoarseness, and airway obstruction, frequently leading to diagnostic confusion with asthma, chronic obstructive pulmonary disease, or endobronchial malignancy. We report an atypical case of a 41-year-old female with primary tracheobronchial amyloidosis. Computed tomography demonstrated diffuse bilateral thickening of the tracheal wall with sparing of the posterior wall with stippled calcifications in the tracheobronchial tree. Radiotherapy was employed as management, leading to stable disease with no signs of progression or regression. This case highlights the diagnostic challenges posed by uncommon imaging features, such as posterior wall sparing, and underscores the importance of integrating multimodality imaging with laboratory and pathological correlation. Early recognition of characteristic and atypical imaging findings is essential for accurate diagnosis and management of this rare but potentially treatable entity.
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