[Pigmented villonodular synovitis of the temporo mandibular joint. Differential diagnosis and therapy]

For the temporomandibular joint (TMJ), functional disorders are common but tumors and tumor like lesions are rare, although these are often mistaken for functional ailments. Early examination by computed tomography or, as a method of choice, magnetic resonance imaging is recommended in case of persisting TMJ problems. Pigmented villonodular synovitis (PVNS) is a rare benign but locally destructive fibrohistiocytic lesion originating in synovial tissue. Involvement of the temporomandibular joint is extremely rare, with the average age of patients being 44.6 years. This paper reports on a 13-year-old patient with diffuse PVNS involving the middle ear and middle cranial fossa. The treatment of choice involves wide local excision and reconstruction of the temporomandibular joint with long-term follow-up. Pathogenesis and differential diagnosis are discussed.