Aggressive laryngeal fibromatosis: A case report and brief review

We describe a rare case of aggressive fibromatosis of the larynx occurring in a 72-year-old man. The lesion manifested with complaints of dyspnea and stridor. A computerized tomographic scan of the neck revealed irregular polypoidal growth invading right vocal cord, crossing midline. A malignant tumor was suspected. Although several laryngoscopy-driven biopsies were negative for malignancy, total laryngectomy was done since the lesion was not deemed amenable to conservative therapy. Tissue sections featured a moderately cellular lesion composed of spindle cells with bland, elongated nuclei, enmeshed in a variably collagenized ground substance. The spindle cells were immunopositive for vimentin and negative for cytokeratins, PGP-9.5, smooth muscle antigen, CD68, desmin, and S-100 protein and Ki-67 of 4%. No further therapy was administered. Based on the available literature, our data confirm that aggressive laryngeal fibromatosis in adult patients is a locally infiltrating disease. Total laryngectomy with clear margins is needed as to avoid the high risk of local recurrence.