Malignant epithelioid schwannoma is a very rare disease originating from the Schwann cells. It is a subtype of malignant schwannoma. The limbs are the main location of this entity. In our case study, the inferior alveolar nerve was involved in the mandible. The prognosis for patients with this disease is poor, because metastasis occurs in more than 50%. Clinical and histological features and treatment in a 42-year-old man are discussed in detail. In addition, a short review of the literature and treatment recommendations are given.
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