Malignant tumors of the temporal bone are very rare, and poorly differentiated sarcoma of temporal bone having not been reported yet. We report the case of a 69-year-old man with poorly differentiated sarcoma of the temporal bone whose symptoms were progressive hearing loss and otorrhea. The tumor was removed using craniotomy and subtotal petrosectomy by neurosurgeon and otologic surgeon consecutively. Diagnosis was established from histologic examination of surgical specimens. During follow-up, however, the tumor was recurred at left petrous apex region 2 years after operation. The patient was treated with radiation therapy with Cyberknife. No recurrence was observed on follow-up MRI imaging until three and half year after radiation therapy. The treatment options of this lesion are discussed with review of literature.
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