Unusual fibrosclerotic lesion of the laryngotracheal complex presenting as subglottic stenosis

We present a case report that describes the pathology, presentation, and management complexities of an unusual, destructive fibrosclerotic lesion of the laryngotracheal complex. An otherwise healthy 21-year-old man presented with a 1-year history of progressive shortness of breath and stridor. The initial examination revealed a 3-cm, grade III subglottic stenosis. Nodular fibrosis of the strap muscles, laryngotracheal cartilages, and trachea was evident. Biopsies revealed dense peritracheal desmoplastic reaction with focal erosion of cartilage. However, features diagnostic for relapsing polychondritis, desmoid tumor, or orbital pseudotumor were absent. The disease progressed to involve severe stenosis and thickening of the trachea and main stem bronchi. Surgical and medical management of this unusual fibrosclerotic lesion did not ameliorate the disease process, but a recent encouraging response to tamoxifen citrate has been observed with improvements in vocal fold motion and activity levels. Prognosis and management experience for this unknown pathologic entity are absent in the literature. In this case, diffuse disease progression occurred despite surgical and medical management, but has been halted by tamoxifen therapy. The prospect of a durable response and disease remission is unknown.