Pigmented villonodular synovitis of the temporomandibular joint with intracranial extension: a case and literature review

Abstract Pigmented villonodular synovitis (PVNS) is a benign, yet locally aggressive proliferative lesion most commonly found in joints of the long bones; it rarely presents in the temporomandibular joint (TMJ). The authors report a case of PVNS involving the TMJ and review similar cases reported in the English literature. This is the first case of PVNS with long-term follow-up of 11 years to include imaging studies. A 36-year-old male with symptoms suggestive of a temporomandibular disorder (TMD) presented with a progressive preauricular/parotid swelling and restricted mandibular range of motion. Imaging suggested a lesion with an aggressive course due to tissue displacement and destruction proximal to the lesion. A multidisciplinary team performed complete excision of the lesion with immediate reconstruction. PVNS often presents with similar symptoms to a TMD, but must be distinguished from symptomatic TMD as it can be highly destructive. Owing to its aggressive nature and potential for recurrence, complete and early extirpation and long-term follow-up with advanced imaging is indicated.