Department of Otorhinolaryngology - Head and Neck Surgery

Case Study from our Complex Cases Series

Michael J. Ruckenstein MD, MSc, FACS, Professor of Otorhinolaryngology - Head and Neck Surgery
Jason A. Brant, MD, Assistant Professor of Otorhinolaryngology – Head and Neck Surgery

Mr. S, a 46-year-old man, presented to Penn ENT with slowly progressive, unilateral hearing loss. On examination, he was found to have a mass filling his external auditory canal. Imaging revealed an expansile lesion of the skull base centered on the temporomandibular joint (Figure 1). The lesion eroded the temporal bone into the external auditory canal, expanded into the middle cranial fossa displacing the temporal lobe, and expanded laterally into the temporalis muscle.

Biopsy and imaging were diagnostic for pigmented villonodular synovitis (PVNS) of the temporomandibular joint. PVNS, also known as an intra-articular giant cell tumor, is a proliferative process of the synovium that is thought to be neoplastic. It is extraordinarily rare in the temporomandibular joint, with fewer than 100 reported cases.

Treatment of PVNS is surgical excision, which in this case involved a multidisciplinary team including neurosurgery and oral surgery and was led by Drs. Bert O'Malley and Michael Ruckenstein. Reconstruction was performed by Dr. Ara Chalian using an anterolateral thigh free flap. The patient recovered well from the surgery and is now more than two years out without any evidence of recurrence of his disease.

PVNS
Figure 1. Expansile pigmented villonodular synovitis (PVNS) of the temporomandibular joint involving the external auditory canal and middle cranial fossa, with displacement of the temporal lobe and lateral expansion into the temporalis muscle.

Discussion

The complex nature of cases such as this highlight the breadth of care available at the Department of Otorhinolaryngology - Head and Neck Surgery at the University of Pennsylvania. Within the department, expertise in skull base surgery, neurotology, and reconstructive surgery allowed for safe and effective surgical treatment. Success in cases like this also rely on the expertise of related specialties such as neuroradiology, pathology, neurosurgery, and oral surgery.

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