Pigmented villonodular synovitis of the temporomandibular joint: a report of two cases.

Abstract

Pigmented villonodular synovitis is a benign but locally destructive disease that originates in the synovial membranes of the joints. It is a proliferative disorder of unknown etiology, and it is usually monarthric. Approximately 80% of cases involve the knee; the hip, ankle, foot, hand, elbow, and shoulder account for most other cases. Pigmented villonodular synovitis in the temporomandibular joint is rare. When it does occur; its features include preauricular swelling, trismus, and symptoms of temporomandibular joint dysfunction. It can be diagnosed by, a combination of the history, clinical examination, characteristic radiologic findings, and fine-needle aspiration or biopsy results. Wide local excision, including the involved bone, and a total synovectomy are advocated because the lesion can recur if it is not adequately excised. We report two new cases of pigmented villonodular synovitis of the temporomandibular joint, and we review the literature on this subject.

Introduction

Pigmented villonodular synovitis is a benign disease of unknown etiology. It arises from the synovial membranes of the joints, bursae, and tendon sheaths. It represents a reactive process of the synovial tissue, it usually involves the knee or other major joint, and it is usually monarthric. Although pigmented villonodular synovitis is histologically benign, it can be a very aggressive lesion, capable of causing extensive bone destruction and capable of widespread infiltration of surrounding tissues. Early diagnosis and treatment are of obvious importance.

Reports of pigmented villonodular synovitis in the temporomandibular joint are rare. When the disease does occur there, it usually arises as a preauricular mass, and it is frequently mistaken for a parotid gland lesion. Symptoms of temporomandibular joint dysfunction are often present, but they can be subtle. Symptoms include painful mastication, clicking, trismus, malocclusion, and deep pain in the joint.

Radiologic evidence of bone destruction is frequently observed, and when it is seen in association with a preauricular mass, it should suggest the disease. Plain radiographs, computed tomography (CT), and magnetic resonance imaging (MRI) have all been described as useful in the diagnosis. Histologically, the lesion features villose hyperplasia, multiple epithelial layers of the synovial lining, and increased vascularity. Multinucleated giant cells and populations of spindle cells are also present.

A review of the literature and careful analysis of the clinicopathologic features of pigmented villonodular synovitis of the temporomandibular joint reveal that the vast majority of reported cases were of the extra-articular variant of this disease, which is associated with more aggressive local infiltrative behavior and a higher rate of local recurrence than is the localized type. Surgical excision is the preferred mode of treatment. Although cosmesis is a consideration, radical excision, including the involved bone, is mandatory to prevent recurrence.

In this article, we report two new cases of pigmented villonodular synovitis of the temporomandibular joint, and we review the previously reported cases. We also discuss its clinical appearance, radiologic and histologic characteristics, and management.

Case reports

Patient 1. A 42-year-old Indonesian man, a welder, came to us with complaints of hearing loss in the right ear of 4 months' duration, clicking in the temporomandibular joint during mastication, and painless preauricular swelling. He had no significant medical history. An audiogram revealed a mild to severe conductive hearing loss. Physical examination revealed that an expansile, pink, glistening mass had nearly occluded the external auditory canal and that a firm preauricular mass had extended from the angle of the jaw to the zygoma. The patient's facial nerve function was intact.

CT demonstrated that a large soft-tissue mass had eroded the mandibular condyle, zygomatic arch, and inferior anterolateral aspect of the petrous bone, with intracranial extension and compression of the temporal lobe (figure 1). The temporomandibular joint was noted to be abnormally wide. MRI revealed a low T1 and T2 signal intensity with some enhancement of the tumor--again with involvement of the temporomandibular joint, infratemporal fossa, and skull base.

[FIGURE 1 OMITTED]

A biopsy was taken through the ear canal. Pathologic examination identified round and spindle-shaped fibrohistiocytic cells and numerous multinucleated giant cells. Areas of abundant hemosiderin pigment and cleft-like pseudoglandular spaces were seen (figure 2). Based on these findings, a diagnosis of pigmented villonodular synovitis was established.

[FIGURE 2 OMITTED]

The patient was taken to the operating room, where exploration revealed that the tumor had filled the external auditory canal and glenoid fossa, eroded the floor of the middle fossa, and extended across the skull base to the foramen lacerum. A wide local excision of the tumor--including a partial mandibulectomy, excision of the zygomatic arch and the contents of the infratemporal fossa and transmastoid middle fossa, and infratemporal removal of the tomur--was accomplished. The patient's recovery was uneventful, and he exhibited no evidence of recurrence at the 3-year follow-up.

Patient 2. A 33 year-old man, a teacher, sought evaluation for a 2-year history of a slowly enlarging right preauricular mass, which had caused deep pain and trismus. Examination revealed the presence of a firm, immobile, 3 x 3-cm preauricular mass. Fine-needle aspiration was performed, and cytologic evaluation identified epithelioid and spindle-shaped fibrohistiocytic cells as well as numerous multinucleated giant cells (figure 3). Hemosiderin was seen in the lesion. These findings were deemed consistent with pigmented villonodular synovitis.

[FIGURE 3 OMITTED]

MRI revealed an enhancing lesion that was isointense on TI- and T2-weighted imaging. The mass was centered on the temporomandibular joint, and it had eroded the lateral margin of the mandibular condyle. The radiologic differential diagnosis included pigmented villonodular synovitis, synovial sarcoma, chondroma, osteochondroma, or a parotid lesion. On surgical exploration, we noted that a pigmented mass had filled the right temporomandibular joint and become attached to the lateral aspect of the mandibular condyle. Wide local excision was performed, including a partial mandibulectomy and excision of the joint. The patient had an uneventful recovery and remained without evidence of recurrence 2 years postoperatively.

Discussion

Although Chassaignac (1) was the first to describe the lesion in 1852, it was a landmark article by Jaffe et al (2) in 1941 that introduced the term pigmented villonodular synovitis. Nearly all cases are found in the large joints; approximately 80% occur in the knee. (2) Other sites include the hip, ankle, foot, hand, elbow, and shoulder. Pigmented villonodular synovitis of the temporomandibular joint is rare.

In 1973, Lapayowker et al were the first to describe pigmented villonodular synovitis in the temporomandibular joint (in two patients). (3) Since then, 22 single case reports have been published in the literature. (4-25) A review of these reports reveals that patients ranged in age from 10 to 70 years and that there was no predisposition as to sex or side. The duration of symptoms at the initial evaluation ranged from 3 weeks to 4 years; in most cases, patients had a preauricular mass, trismus, pain, and temporomandibular joint symptoms. Occasional hearing loss as a result of disruption of the middle ear or external auditory canal has also been reported. (3,5,15,20,25)

Nearly all of the earlier cases of temporomandibular pigmented villonodular synovitis were preoperatively presumed to be parotid lesions. In the more recent reports, the correct preoperative diagnosis was made more often, based on characteristic radiologic findings, fine-needle aspiration or biopsy, and previous experience with the disease. In nearly all cases, bone erosion was evident on preoperative imaging. Likewise, nearly all cases were of the extra-articular variant. (26)

CT clearly reveals areas of bone erosion and cyst formation. CT also defines the extent of the tumor well because (1) the tumor has lower attenuation values than does the surrounding muscle and (2) CT enhances the thickened synovium and areas of high attenuation caused by iron deposits. (25) Even so, MRI is thought to be the best radiographic method for judging the extent of the tumor. Pigmented villonodular synovitis typically demonstrates low signal intensity because of the presence of hemosiderin, and bony erosion is typically seen. Evidence of pigmented villonodular synovitis on MRI is strongly suggestive of the diagnosis, but it is not pathognomonic because of the somewhat variable composition of these tumors. (27) MRI does not assess the vascularity of the lesions well, and for this reason, some physicians have turned to angiography. The angiographic features of pigmented villonodular synovitis are nonspecific, but these tumors are typically highly vascularized, with arteriovenous shunting, irregular vessels, and dense tumorblush. (25)

References

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From the Division of Otolaryngology-Head and Neck Surgery, Department of Surgery, Loma Linda (Calif.) University.

Reprint requests: Paul A. Martin, MD, Division of Otolaryngology-Head and Neck Surgery, Loma Linda University, 11234 Anderson St., Loma Linda, CA 92354. Phone: [phone]; fax: [phone]; e-mail: [e-mail]

Originally presented at the annual scientific meeting of the Southern California Chapter of the American College of Surgeons; Jan. 22, 2000; Huntington Beach, Calif.