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Pigmented villonodular synovitis of the temporomandibular joint
Abstract:
Pigmented villonodular synovitis ( PVNS ) is a benign disease of unknown etiology arising from the synovial membranes of joints, bursae, and tendon sheaths. Though histologically benign, it is a very aggressive lesion, capable of bone destruction and widespread infiltration of surrounding tissues. It is a rare disease, usually confined to large joints, particularly the knee. Its propensity for recurrence is emphasized in this report. Involvement of the temporomandibular joint (TMJ) is unusual. It most commonly presents in the TMJ region as a left pre-auricular mass. Symptoms of TMJ dysfunction are usually present, but may be subtle. These may consist of painful mastication, clicking, trismus, malocclusion, and deep pain in the TMJ. Radiological evidence of bone destruction is frequently present, and together with the pre-auricular mass, should suggest the diagnosis. Surgical excision is the preferred mode of treatment. While cosmesis is a consideration, radical excision, including involved bone, is mandatory to prevent recurrence. The effect of radiotherapy on this disease in the head and neck region is documented.
Insights
Pigmented villonodular synovitis (PVNS) is a rare, aggressive joint disease. Early diagnosis and radical surgical excision are crucial for preventing recurrence, especially in unusual locations like the temporomandibular joint.
Area of Science:
- Orthopedics
- Pathology
- Oncology
Background:
- Pigmented villonodular synovitis (PVNS) is a rare neoplastic proliferation of the synovium.
- PVNS is characterized by aggressive local invasion and a high recurrence rate, despite its benign histological nature.
Observation:
- PVNS typically affects large joints, with the knee being the most common site.
- Temporomandibular joint (TMJ) involvement is uncommon, often presenting as a pre-auricular mass.
- TMJ dysfunction symptoms include masticatory pain, clicking, trismus, and malocclusion.
Findings:
- Radiological evidence of bone destruction and a pre-auricular mass are key diagnostic indicators for TMJ PVNS.
- Radical surgical excision, including affected bone, is the primary treatment to prevent recurrence.
- The efficacy of radiotherapy in head and neck PVNS cases has been documented.
Implications:
- Accurate diagnosis and aggressive surgical management are vital for optimal outcomes in PVNS patients.
- Increased awareness of rare presentations, such as TMJ involvement, can lead to earlier intervention.
- Further research into PVNS etiology and treatment modalities may improve long-term prognosis.
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