Leiomyosarcoma involving the larynx is extremely rare and may be difficult to diagnose. Because of the rarity of this tumor, little information exists on its long-term follow-up and optimal management. We present a review of the literature and report on a patient with leiomyosarcoma of the larynx treated with surgery and postoperative irradiation. In addition, the diagnosis and treatment of leiomyosarcoma are discussed. At six months’ follow-up the patient showed no signs of local recurrence but had developed metastases to both lungs.
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