Extra-articular pigmented villonodular synovitis of the temporomandibular joint

Pigmented villonodular synovitis, a benign but locally destructive fibrohistiocytic proliferative lesion involving tendon sheaths, bursae and diarthrodial joints, is distinctly rare in the temporomandibular joint. We report one such case occurring in a 42-year-old housewife who presented with a progressively enlarging right zygomatic mass for six months. On exploration, an orange-brown firm mass, 5 x 3 x 2 cm, was seen adherent to the lateral aspect of the capsule of the right temporomandibular joint, and eroding into the inferior aspect of the right temporal bone and part of the mandibular condyle. The mass was completely excised. Pathological examination showed features typical of those of pigmented villonodular synovitis and the lesion was entirely extra-articular in location. The patient remained well with no evidence of local recurrence two years after operation. Review of the literature and careful analysis of the clinicopathological features showed that the vast majority of the reported cases of pigmented villonodular synovitis of the temporomandibular joint belonged to the extra-articular variant, which is associated with a more aggressive local infiltrative behaviour and higher rate of local recurrence than the localized type. The recommended treatment for this condition is therefore wide local excision, aiming to remove the lesion as completely as possible without producing severe disability for the patient.