Background: Tumors of smooth muscle origin are rare in the upper aerodigestive tract, due to the paucity of smooth muscle in the area. A review of the literature revealed 34 reported cases of leiomyoma, 9 cases of leiomyosarcoma, and only 2 cases of epithelioid leiomyoma arising in the larynx.
Methods: A case report of an epithelioid leiomyosarcoma arising in the right piriform fossa of a 38-year-old man is presented.
Results: The tumor was treated with conservative surgical resection and postoperative radiotherapy. There was no evidence of locoregional recurrence 2 years postsurgery, and laryngeal function was preserved.
Conclusions: To our knowledge, this is the third case of a laryngeal epithelioid smooth muscle tumor to be reported in the world literature and the only documentation of an epithelioid leiomyosarcoma arising in the larynx.