Pigmented Villonodular Synovitis of the Temporomandibular Joint: A Unique Presentation

An 85-year-old man was referred to the Otolaryngology-Head and Neck Surgery clinic in February 2013 for left ear pain associated with a small lesion with intermittent bleeding. He had no other systemic or neurologic symptoms. This was believed to be chondrodermatitis nodularis chronica helicis. One year later, the patient was rereferred for a preauricular mass on the ipsilateral side (Fig. ​(Fig.1)1) with an inconclusive fine needle aspirate. Fig. 1. Clinical image of the preauricular mass over the left temporomandibular joint (arrow). From a symptomatic perspective, the patient had ear pain and intermittent headaches but denied any facial numbness or weakness, weight loss, fever, anorexia, trismus, or impaired hearing. The patient had a positive history of trauma to the area as he was struck with a large piece of lumber 7 years before. Computed tomography scan and magnetic resonance imaging of the face and skull base showed a destructive process with intracranial extension centered at the left temporomandibular joint (TMJ) with hypertrophy and enhancement of synovium. The lesion was peripherally calcified and heterogeneous (Fig. ​(Fig.2).2). The findings on imaging correlated with a differential diagnosis of synovial chondromatosis, chondrosarcoma, or pigmented villonodular synovitis (PVNS). A repeated fine needle aspirate was performed, which showed no evidence of malignancy and findings most compatible with PVNS. Fig. 2. Coronal computed tomography scan of the face/skull base showing a peripherally calcified and heterogeneous process centered at the left temporomandibular joint (arrow). This patient’s case was discussed at Head and Neck Tumor Board. Because of radiologic features not in keeping with the suspected pathologic diagnosis, it was felt that an open biopsy was the best way to proceed. The patient underwent an uneventful transfacial TMJ biopsy through a preauricular incision. Pathology from several pieces of the mass showed a lobulated mildly atypical cartilaginous proliferation associated with synovium with reactive changes, including chronic inflammation. The open biopsy pathology ruled out chondrosarcoma. This pathology coupled with the history of trauma and intracranial extension confirmed the diagnosis of PVNS. The patient did not receive any further surgical intervention given his age and that the diagnosis was most in keeping with a PVNS of the TMJ. The patient is since doing well and is being followed by the Head and Neck Surgery team; he receives magnetic resonance imagings every 6 months, and there has been no interval growth with conservative management over the last year.