Pigmented villonodular synovitis of the temporomandibular joint with intracranial extension: A case series and systematic review

Background Pigmented villonodular synovitis (PVNS) is a rare proliferative disorder of the synovial membrane. PVNS generally affects large joints but occasionally involves the temporomandibular joint (TMJ), with occasional extension into the middle cranial fossa. The purpose of this study was to report our experience with PVNS along with a focused literature review. Methods Patients with PVNS of the TMJ treated at the University of California - San Francisco from 2007 to 2013 were reviewed. A PubMed search was performed to identify additional cases. Results Five patients underwent surgical resection, with 1 recurrence at 61 months. A literature review identified 58 patients, 19 of which had intracranial involvement. Interestingly, intracranial extension was more common in men. Intracranial extension was not associated with an increased rate of recurrence. Conclusion PVNS of the TMJ is a rare entity associated with excellent outcomes, even with intracranial extension. Management should consist of maximal resection, with radiotherapy reserved for extensive or recurrent lesions.