Aggressive fibromatosis of the larynx: Report of a new case in an adult patient and review of the literature

We describe a case of aggressive fibromatosis of the larynx occurring in a 75-year-old man. The lesion manifested with voice hoarseness and swallowing difficulty. A computerized tomographic scan of the neck revealed distortio n of the glottic profile. A malignant tumor was suspected. Although a laryn goscopy-driven biopsy was non-diagnostic, total laryngectomy was done, sinc e the lesion was not deemed amenable to conservative therapy. Grossly, the glottic rim was infiltrated by a hard, grey-white tissue showing a tentacul ar outline. Tissue sections featured a moderately cellular lesion composed of spindle cells with bland, tapered nuclei, enmeshed in a variably collage nized ground substance. Delicate spindle cell fascicles sur rounded the nat ive submucosal seromucous glands and had invaded the thyroid cartilage and the thyroid gland as well. The spindle cells were immunopositive for actins and vimentin, and negative for keratins, epithelial membrane antigen, desm in, and S-100 protein. No further therapy was administered. Periodic follow -up visits were negative. The patient died 5 years after surgery of myocard ial infarction with no clinical evidence of lesion recurrence. Based on the available literature, our data confirm that laryngeal fibromatosis in adul t patients is a locally infiltrating and progressive disease. Total larynge ctomy with clear margins is needed as to avoid the high risk of local recur rence.