Pigmented Villonodular Tenosynovitis of the Temporomandibular Joint—A Case Report

Background: Pigmented villonodular synovitis is a rare condition typically affecting the major joints and the involvement of the temporomandibular joint is rare. Although, it is histologically benign, it can be locally aggressive, capable of extensive bone destruction. The disease has aggressive behavior and a high incidence of recurrence (40% 60%). Case report: A 35 years old male presented with history of unilateral hearing loss and otalgia of six month duration. On examination, there was a soft bulge in the superior wall of external auditory canal. Contrast enhanced CT scan showed soft tissue density occupying the right external auditory canal and bulging into the middle ear cavity, displacing the intact ossicular chain medially with evidence of destruction of the posterior wall of the external auditory canal, glenoid fossa, mastoid bone and the scutum. The squamous part of the right temporal bone showed evidence of lateral bowing with discontinuity. Surgical resection was done through post aural approach. Histopathological examination revealed pigmented villonodular tenosynovitis which stained positive for CD 68. Post operatively patient was evaluated clinically and with radiological modalities which showed healthy mastoid cavity and no increase in post operative granulations on magnetic resonance imaging. Conclusion: Pigmented villonodular tenosynovitis of temporomandibular joint is a rare entity. Due to the aggressive behavior of the lesion, radical or aggressive surgical resection should be done. Long term follow up with radiological modality should be done due to the high recurrence rate.