Low-grade myofibroblastic sarcoma is an uncommon sarcoma with myofibroblastic differentiation. It occurs in a wide variety of sites and has a predilection for the head and neck region. Biologically, low-grade myofibroblastic sarcoma has a propensity for local recurrence and is associated with a low risk of metastatic spread. Histologically, it can mimic a variety of different types of benign and malignant processes and often requires immunohistochemical analysis for its accurate identification. This report describes a case and discusses the differential diagnosis of a low-grade myofibroblastic sarcoma that arose in the larynx of a 69-year-old woman with a history of metastatic skin melanoma. To the best of the authors’ knowledge this is the first description in the English literature of low-grade myofibroblastic sarcoma originating in the larynx.
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