Diffuse variant tenosynovial giant cell tumor of the temporomandibular joint: Report of a case

991

OMURA ET AL J Oral Maxillofac Surg 56:991-996, 1998

Diffkse Variant Tenosynovial Giant Cell Tumor of the Temporomandibular Joint: Report of a Case Susumu

Omura, DDS, PhD, * Nobuyuki Mizuki, DDS, PbD, f Hiroki Bukawa, MD, DDS, PbD,# and Kiyohide Fujita, MD, DDS, PbDJ

Tenosynovial giant cell tumor (TSGCT) and pigmented villonodular synovitis (FVNS) are both regarded asa common family of synovial lesions because of their histologic similarity. Both consist of closely compacted polyhedral or rounded cells, variable numbers of giant cells, foamy macrophages, and hemosiderin deposition, intermingled with hypocellular collagenized zones where the cells tend to be spindled.1-3 To our knowledge, this is the first case report of dilYuse variant TSGCT of the temporomadibular joint (‘Ml. This type of tenosynovial lesion haspreviously been referred to as xanthoma, xanthogranuloma, giant cell tumor, xanthomatous giant cell tumor, myeloplaxoma of the tendon sheath with synovial and bursal lesions, chronic hemorrhagic villous synovitis, fibrohemosideric sarcoma, and sarcoma fusogigantocellulare, as well as by other complicated names. Because of variable clinical features and cellular polymorphism, cells, the nomenclature has been confusing for the past century. Clinicopathologic grouping of these lesions was first proposed in 1941 by Jaffe et al? This concept was further refined in 1995 by Enzinger and Weiss.3 The nomenclature used today primarily reflects the anatomic location, macroscopic appearance, and clinical behavior, all of which show dramatic variation. TSGCT presents in both localized and diffuse forms.‘,3 Localized TSGCT, alternatively known as

Received from the Department of Oral and Maxillofacial Surgery, School of Medicine, Yokohama City University, Yokohama, Japan. *Assistant Professor. tAssi$tant Professor. *Instructor. §Professor. Address correspondence and reprint requests to Dr Omura: Department of Oral and Maxi!.lofacial Surgery, School of Medicine Yokohama City University, 3-9 Fukuura, Kanazawa-ku, Yokohama 236, Japan. o 1998 American Association of Oral and Maxillofocial 0278.2391/98/56080015$3.00/0

Surgeons

nodular tenosynovitis or giant cell tumor of the tendon sheath, is one of the most common tumors of the hands and feet. In comparison, d8use TSGCT is less common and shows certain clinical differences. Unlike localized TSGCT, the lesion has a tendency to occur in younger individuals, present as a less welldefined soft tissue mass, and show locally aggressive growth and a high recurrence rate. Diffuse TSGCT alYects relatively large joints, typically the knee and ankle.* PVNS is characterized by frondlike, villous formations that fill the articular space, and abundant intracellular or extracellular hemosiderin deposition. l PVNS can be regarded as the intraarticular counterpart of d&se TSGCT, and its anatomic distribution parallels that of diffuse TSGCT. PVNS has been reported to occur in the TMJ,5-7but no casesof diffuse TSGCT of the TMJ have been reported in the literature.

Report

of Case

An B-year-old Japaneseman presentedwith a IO-month history of left TMJ pain andgraduallyprogressiveinability to open hismouth. Hishistory includedinjury to the mandible incurred in a fight 5 years previously. On examination, a tender, diffuse swelling was noted in the left preauricular region, and his maximum interincisal opening was 20 mm. Panoramic radiography and tomoradiography of the TMJ

showed resorption of the left condyle and a deepened glenoid fossa, resulting in a widened joint space. Computed tomography (CT) scans showed pressure resorption of the cortex of the left glenoid fossa and bony erosion of the medial aspect of the left condyle (Fig 1). Magnetic reso-

nance images(MRIs) showed a relatively well &cumscribed, rounded, soft tissuemassmeasuring3 X 3.5 cm around the left condyle, which was resorbed(Fig 2). The mass had expanded in all directions, but predominantly along the left external pterygoid muscle (Fig 3), half of which had been involved by the mass Figs 3, 4). On T,-weighted images, the signal intensity of the mass was equal to or slightly lower than that of the muscle (Figs 2, 3). On Tz-weighted images, the mass had a heterogeneous appearance, with areas of markedly high signal intensity bordered by rims of decreased signal intensity and areas of slightly lower signal intensity than that of the muscle (Fig 4). The areas of markedly high signal intensity appeared to be

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TENOSYNOW

FIGURE external

FIGURE 1. Axial CT scan showing pressure resorption of left glenoid fossa (arrowheads) and less well-defined erosion on the medial aspect of the left condyle (arrow).

posterior cortical

wall bone

consistent with synovial fluid. A 99mTc bone scan showed abnormally intense uptake involving the left TMJ. The lesion was exposed through a preauricular incision (Fig 5) and it appeared to lack a fibrous capsule. The enlarged joint cavity contained a brown serosanguineous fluid and no articular disc was discernible. An intraoperative biopsy was performed for frozen sectioning, which was diagnosed as a possible tenosynovial giant cell tumor. Consequently, total excision of the soft tissue mass was performed. After bone curettage of the condyle and the glenoid fossa, a temporal muscle and fascia flap was inserted into the joint space as an interpositional graft to prevent TMJ ankylosis. The postoperative course was uneventful, and the TMJ pain and limited mouth opening were completely

FIGURE

2. Coronal Tl-weighted MRI showing left condyle. Signal intensity of lesion is basically slightly lower than that of skeletal muscle.

soft tissue mass around low and equal to or

3. Axial pterygoid

Tj-weighted muscle.

GIANT CELL TUMOR

MRI

showing

OF THE TMJ

involvement

of half

of

resolved. During a 2-year follow-up, no recurrence was observed. Microscopic examination of the surgical specimen showed that the lesion predominantly consisted of rounded to polygonal mononuclear cells with sparse eosinophilic cytoplasm and large round or oval nuclei, and a mononuclear cell inflammatory infiltration (Figs 6, 7). In hypocellular collagenized zones, the cells tended to be spindled (Fig 6). Hemosiderin-laden macrophages (Fig 8) and multinucleated giant cells showing moderate variation in size, shape, and number of nuclei were focally present (Figs 6, 7). The intraarticular component containing synovial fluid was lined with a thickened layer of rounded to spindled synovial-like

FIGURE 4. Axal Tz-weighted MRI showing lesion with heterogeneous appearance. Areas of markedly increased intensity (*) with a rim of decreased signal (arrows) appear para-articular synovial fluid collection.

septated signal to be a

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OMURA ET AL

tion of EnzingerandWeiss,3the lesioncould be regardedas a diffusevariant of TSGCT.

Discussion

FIGURE preauricular

5.

Total incision.

excision

of

the

lesion

was

performed

through

cells.This thickened layer occasionallyformed microscopic villous projectionssimilarto thoseof PVNS(Fig 9). Basedon histologic findings,the lesionwas diagnosedas TSGCT of the TMJ. The lesion lacked an evident fibrous capsule,and radiographicfindings, including CT scansand MFUs,indicated locally aggressivegrowth and predominantly extraarticular extension.According to the classifica-

~s~~‘xo~i~~o~~~~~~r~~hp~~ lygonal mononuclear cells admixed with an inflammatory mononuclear cell infiltration. In the hypocellular collagenized zones, cells tend to be spindled (Hematoxylin-eosin stain, original magnification X401.

The clinical features of this case are typical of di@useTSGCT except for the anatomic location. The lesion occurred in a young person with a history of some trauma. The initial diagnostic possibilities included synovial cyst, ganglion, degenerative arthritis, and neoplastic lesion. Of the four previously reported casesof PVNS of the TMJ, two caseswere preoperatively diagnosed asa parotid tumor.5,7 On axial CT scans,the posterior wall of the glenoid fossa was markedly concave because of pressure resorption, whereas less defined bone erosion was evident at the inner aspect of the condyle where the tendon of the external pterygoid muscle was attached. Typical osteal changes described for diffuse TSGCT or PVNS in other major joints include smoothly marginated cortical bone erosion adjacent to the joint space and diffuse osteoporotic degeneration at points of synovial attachment.3 The decreased signal intensity on both T2- and T,-weighted images is an uncommon feature of some soft tissue massesin the hands or feet, and suggests the diagnosis of TSGCT.8,9Compared with the signal intensity of the muscle, TSGCT is usually isointense on Tr-weighted images. However, on T,-weighted images, the signal intensity varies considerably from

994

TENOSYNOVLAL

GIANT

CELL

TUMOR

OF THE

TMJ

FIGURE 7. Photomicrograph showing focal multinucleated giant cells with moderate variation in size, shape, and number of nuclei (Hematox$in-eosin stain, original magnification X400).

hypointense to hyperintense relative to the muscle and often appears to be heterogeneous.9 A mixture of high and low signals on T,-weighted images reflects the histology of the lesion. Areas of marked high signal

FIGURE 8. Photomicrograph showing macrophages containing granules of hemosiderin (Hematox lin-eosin stain, original magniica+ion X400].

intensity on T,-weighted images with hypointensity on T,-weighted images correspond to synovial fluid, and areas with hypointensity to isointensity relative to the muscle on both T,- and T,-weighted images

OMURA

ET AL

FIGURE

9. Photomicrograph showing the intra-articular component of the lesion lined with a thickened layer of rounded to spindled synovial-like cells. This thickened layer forms occasional villous projections similar to those of PVNS [Hematovlin-eosin stain, original magnification X40).

correspond to areasof fibrosis or hemosiderin deposition.*-lo The amount of hemosiderin in TSGCT lesions varies considerably, and it is sometimes absent. If less hemosiderin is present, the signal intensity is more likely to increase.9 MRI findings in the current case were consistent with TSGCT in the extremities. When a soft tissue massinvolving the TMJ shows low signal intensity equivalent to that of muscle on T,-weighted images, and a heterogeneous appearance on TZweighed images, diffuse TSGCT should be considered in the differential diagnosis. Most cases of diffuse TSGCT can be regarded as extraarticular extensions of the primary intraarticular process, and a purely extraarticular origin of diEuse TSGCT is rare.l,s,* Similarities between diffuse TSGCT and PVNS are observed with respect to age, location, clinical presentation, and symptoms. In many instances, distinguishing clearly diffuse TSGCT with an intraarticular component from PVNS is difficult. Thus, the term “PVNS-diEuse” has often been used synonymously with “TSGCT-diffuse.” However, “PVNS” should be restricted to extensive intraarticular involvement, and should not be applied to diEuse extraarticular extension. l PVNS shows excessive pigmented villous formation within the articular space, resulting in the so-called shaggy beard appearance.” The intraarticular villous projections have a great tendency to bleed, leading to obvious brown pigmentation of the PVNS resulting from abundant deposition of hemosiderin.3,8 In the

current case, the articular space containing synovial fluid was lined with a multilayer of cuboidal cells, most likely originating from hyperproliferation of synovial cells. This thickened layer microscopically showed a focal villous pattern, but macroscopically discernible pigmented villous formation in the articular space was not observed. The current case most likely originated from the synovial lining of the TMJ and extended predominantly toward the paraarticular tissue. Arthroscopy appears to be useful for the diagnosis of PVNS. Several authors have reported that frondlike villous formation filling the articular space could be clearly visualized using this imaging method in the knee.*l,12 In the current case, arthroscopy was not performed. However, normal arthroscopic features may not exclude TSGCT with any certainty,‘3 because diffuse TSGCT lacks the grossly discernible pathologic changesin the synovial lining of the joint. Indication for treatment should be inlluenced by the nature of the lesion. The nature of TSGCT and PVNS has been controversial, but recent evidence suggestsa neoplastic rather than inflammatory origin.3 Localized TSGCT is well circumscribed by a mature collagenous capsule, and its surgical removal is relatively easy.‘* In diffuse TSGCT and PVNS, the optimal therapeutic strategy is not yet known. Because PVNS is essentially restricted to the intraarticular space, total or subtotal synovectomy has been advised as the treatment of choice. When the bone is involved,

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HORNER’S

curettage of the lesion is also required. Few studies have examined the treatment of diffuse TSGCT. AbdulKarim et al’ reported that the percentage of S+G2M phase of the cell cycle in the total cell population (termed “proliferative index”) may assist in predicting aggressive biologic behavior and determining the clinical course that should be considered in the management of the patient.l For local control of the lesion, wide excision or resection may be the best choice, but this involves significant morbidity. Despite a high recurrence rate, reported cases of malignant transformation or distant metastasis are rare. Thus, these lesions should be regarded as locally aggressive, but not as malignant neoplasms. Enzinger and Weiss’ postulated that therapy should be based on a desire to remove the tumor as completely as possible without producing severe disability for the patient. The current case was treated in accordance with this guideline. The lesion was excised as completely as possible with preservation of the condyle, and a temporalis muscle and fascia flap was used as an inter-positional graft to prevent TMJ ankylosis. During a 2-year follow-up period, no complaint of disability in the TMJ and no evidence of recurrence have been observed. The patient will be followed long-term, because there can be recurrence after several years. Although radiation therapy has been used for treatment of surgically unresectable lesions, it is not clear whether such treatment can decrease the incidence of recurrence.15

SYNDROME

IN BASILAR

SKULL

FRACTURE

References 1. Abdul-Karim FW, El-Nagger AK, Joyce MJ, et al: Diffuse and localized tenosynovial giant cell tumor and pigmented villonodular synovitis: A clinicopathologic and flow cytometric DNA analysis. Hum Path01 23:729, 1992 2. Jaffe HL, Lichtenstein L, Sutro CJ: Pigmented villonodular synovitis, bursitis and tenosynovitis: A discussion of the synovial and bursal equivalents of the tenosynovial lesion commonly denoted as xanthoma, xanthogranuloma giant cell tumor or myeloplaxoma of the tendon sheath, with some consideration of this tendon sheath lesion itself. Arch Path01 31:731, 1941 3. Enzinger FM, Weiss SW: Soft Tissue Tumors (ed 3). St Louis, MO, Mosby, 1995, p 735 4. Rowlands CG, Roland B, Hwang WS, et al: Diffuse-variant tenosynovial giant cell tumor: A rare and aggressive lesion. Hum Path01 25423, 1994 5. Lapayowker MS, Miller WT, Levy WM, et al: Pigmented villonodular synovitis of the temporomandibular joint. Radiology 108: 313,1973 6. Barnard JDW: Pigmented villonodular synovitis in the temporomandibular joint: A case report. BrJ Oral Surg 13:183, 1975 7. Miyamoto Y, Tam T, Hamaya K: Pigmented villonodular synovitis of the temporomandibular joint. Plast Reconstr Surg 59:283, 1977 8. Jelinek JS, Kransdorf MJ, Utz JA, et al: Imaging of pigmented villonodular synovitis with emphasis on MR imaging. Am J Roentgen01 152:337, 1989 9. Jelinek JS, Kransdorf MJ, Shmoolker BM, et al: Giant cell tumor of the tendon sheath: MR findings in nine cases. Am J Roentgenol162:919,1994 10. Narra VR, Shirkhoda A, Shetty AN, et al: Giant cell tumor of the tendon sheath in the ankle: MRI with pathologic correlation. J Magn Reson Imaging 5:781, 1995 11. Dorfmann H: Arthroscopic detection of synovial disorders. Contemp Orthop 10:19,1985 12. Flandry FC, Hughston JC: Current concepts review: Pigmented villonodular synovitis. J Bone Join Surg Am 69:942, 1987 13. Klompmaker J, Veth RPH, Robinson PH, et al: Pigmented villonodular synovitis. Arch Orthop Trauma Surg 109:205, 1990 14. Glowacki KA, Weiss A-PC: Giant cell tumors of tendon sheath. Hand Clin 11:245,1995 15. Bertone F, Pignatti G, Bacchine P, et al: Miscellaneous synovial lesions. Curr Opin Rheum 2:120, 1990

J Oral Maxillofac Surg 56:996-l 000, 1998

Horner’s Syndrome Secondary to a Basilar Skull Fracture After Maxillofacial Trauma James P. Worthington, MB CbB, BDS, FFDRCS* and Leslie &ape, MB ChB, BDS, FRCS, FUCDS, FFDRCSJ-

*Registrar,

Oral

Dublin. fConsultant,

& Maxillofacial

Oral and Maxillofacial

Surgery, Surgery,

St James’ Christchurch

Hospital, Hospi-

tal, New Zealand. Address

correspondence

and reprint

requests

to Mr

Consultant, Oral and Maxillofacial Surgeon, Christchurch Private Bag 4710, Christchurch, New Zealand. o 1998 American Association of Oral and Maxillofaciul 0278.2391,‘98/5608-0016$3,00/O

Surgeons

Snape: Hospital,

Not uncommonly, patients with maxillofacial injuries have associated head injuries necessitating the involvement of the neurosurgical service. Lim et al’ reviewed 839 patients with facial injuries and found that 5.4% suffered neurosurgical injury requiring consultation. Other reviews have shown up to 51.6% of patients with facial injuries having other associated injuries.2 Haug et al, in a retrospective review of 882 patients with facial fractures, found a 4.4% incidence of related cranial fractures, with the frontal (38%) sphenoid