Pigmented villonodular synovitis (PVNS) is an aggressive proliferative lesion that usually involves the synovial tissues of big joints. To date, there are ∼52 cases of PVNS affecting the temporomandibular joint reported in the English-language literature, about one-third of them exhibiting intracranial involvement. We herein describe an additional case of PVNS of the temporomandibular joint with skull base invasion affecting a 26-year-old male patient and discuss its clinicopathologic features considering previously published cases. Histopathology and imaging evaluation are important for the diagnosis of PVNS, which should be included in the differential diagnosis of preauricular aggressive swellings.
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