Case report

A 63-year-old Caucasian man was referred to the authors’ institution in May 2010 with a mass in the left preauricular area that had been growing for the past 2 years. His general medical history was non-contributory. He denied any antecedent trauma on his face, as well as any locking, clicking or pain, and was only complaining of a tender sensation in the pre-auricular area.

His maximum inter-incisal opening was 42 mm with no deviation to the left side during mandibular excursion. Visual examination detected a mild swelling in the region, which on palpation revealed a remarkable, diffuse mass underlying the cutis. An orthopantomogram showed normal morphology of the head of the left condyle of the mandible. Computed tomography (CT) and magnetic resonance images (MRI) showed normal morphology and location of the left meniscus either with opened or closed mouth and detected no structural bone abnormalities ( Fig. 1 ). They revealed a relatively well circumscribed, rounded mass of left peri-articular soft tissues with a transversal diameter of 21 mm. The medial aspect of the lesion suggested an origin from articular structures though it expanded in all directions with a moderate displacement deep to the temporal lobe of the left parotid. T ₂ -weighted MRI sequences showed marked signal hypointensity throughout the lesion ( Fig. 1 B). Considering the clinical history and the imaging features with no destructive growth, the main clinical differential diagnosis was a parotid gland neoplasm. Cytology using fine needle aspiration was undiagnostic. The patient underwent surgery under general anaesthesia through a pre-auricular approach. Once exposed, the lesion appeared to lack a fibrous capsule and was a brown-reddish mass with a soft and friable texture ( Fig. 2 A ). An intraoperative biopsy suggested a possible DTGCT. Careful examination of the TMJ revealed no evidence of involvement. Consequently, the whole mass was removed as completely as possible preserving TMJ structures and facial nerve branches.

(A) Axial CT scan of the left TMJ showing a round mass growing in peri-articular soft tissues with no invasion of the articular structures (arrows). (B) T2-weighted coronal MRI sequence showing a low signal of the lesion which appears dark (arrows).

(A) Macroscopic aspect of the lesion showing as brown-reddish mass without a fibrous capsule. (B) Photomicrograph of haematoxylin–eosin staining showing mononuclear cells and multinucleated giant cells (arrows) with variations in size, shape and number of nuclei (100×). (C) Immunohistochemical staining presents macrophage-associated antigen CD68 in the cytoplasm of the multinucleated giant cells and mononuclear cells (200×). (D) Pearls staining shows hemosiderinic nature of granules (200×).

Microscopic examination of the specimen showed nests of spindled or epithelioid mononuclear cells infiltrating the muscle and parotid gland involvement with fibrous tissue containing multinucleated giant cells pigmented by granules ( Fig. 2 B). Pearls staining suggested a hemosiderinic nature for these granules ( Fig. 2 D). Immunostaining revealed a HBM ⁻ , MelanA ⁻ , S-100 protein ⁻ pattern, but strong granular cytoplasmic reactivity with CD68 of giant and mononuclear cells ( Fig. 2 C). Ki-67 staining showed a low mitotic index. Based on the clinical, imaging, histologic and immunohistochemical findings the lesion was diagnosed as DTGCT. No additional adjuvant therapy was suggested. The patient developed post-surgical facial paralysis which resolved after 2 months. No recurrence occurred after 12 months of follow-up.

Discussion

The authors describe a purely extra-articular case of TMJ-DTGCT. DTGCT belongs to a family of lesions affecting the synovium historically divided in two groups: PVNS and pigmented villonodular bursitis; and localized nodular synovitis and pigmented villonodular tenosynovitis. The first group included the diffuse form in diarthroidal joints and bursa/tendon synovium, while the second was the exact counterpart with a localized appearance. In the medical literature, localized forms have generally been regarded as GCTTS as they characteristically affect the synovial tendon sheath of the hands and feet. Diffuse forms have mainly been regarded as PVNS, because they often arise from the articular synovium; when the origin appears to be from bursa or tendon sheaths the attribute ‘extra-articular’ has been added. Many authors use the term ‘extra-articular PVNS’ to describe the clinical presence of an extra-articular expansion of the lesion and not because of a primary synovial origin.

This potential misunderstanding has occurred in the TMJ area. In 1995 Enzinger and Weiss introduced the term tenosynovial giant cell tumour and classified it into a diffuse variant (i.e. D-TTGCT) and a localized variant (i.e. GCTTS). They indicated that the diffuse form was a synonym of PVNS with extra-articular origin (i.e. bursae and tendons) with or without involvement of an adjacent joint.

In 1998 Omura et al. reported one case of D-TTGCT of the TMJ and described it as the first case. To date, two other cases have been described, one recently, although its existence had been questioned by Lee et al. in 2000. They thought the case described by Omura et al. was extrarticular PVNS, as Enzinger and Weiss had indicated it was a synonym of D-TTGCT. The present authors point out that the D-TTGCT of TMJ cannot exist as Enzinger and Weiss considered the extra-articular synovial origin of the lesion and not the clinical aspect of extra-articular diffusion, also the TMJ lacks bursae and sliding tendons lined by synovium, unlike the fingers and knee.

The present authors have adopted the nomenclature of the current WHO classification of soft tumours, which considers the diffuse forms as DTGCT, and the localized ones as GCTTS. All these lesions share the common histologic findings of a villous to nodular synovial hyperplasia with proliferating synovial histiocyte-like cells, which are polyhedral, round or spindled, and have variable proportions of lymphocytes, giant cells, foamy macrophages and hemosiderin pigment. Immunohistochemically, the mononuclear cells express CD68. This cellular polymorphism has caused authors to debate whether this condition is a reactive lesion or true neoplasm. Findings of DNA aneuploidy, clonal karyotypic aberrations and up-regulation of telomerase activity as well as of the anti-apoptotic human peptide strongly suggested a neoplastic property for DTGCT. Though rare, malignancy and death from metastasis has been also reported in extra-articular forms. Both could fit the hypothesis given recent observations of an increased expression of colony stimulating factor 1 (CSF1) in synovial cells associated with its gene translocation. CFS1 binds the CSF1 receptor expressed by the synovial cell itself as well as by inflammatory cells, such as histiocytes, lymphocytes and multinucleated cells. The first autocrine effect causes neoplastic proliferation, the second paracrine effect determines the recruitment of inflammatory cells.

All these findings are shared by DTGCT and GCTTS and it has recently been advocated that the denomination of these conditions should be unified.

The authors reviewed all reported cases of giant cell tumour of the TMJ based on the clinical/intraoperative aspect of TMJ involvement. 56 cases were found in the English language literature ( Table 1 ). They all were unilateral. 54 cases (average age 44.0 ± 14.55 years) showed a diffuse pattern of growth and so were recognized as DTGCT. Of these, 50 developed in both extra-articular and intra-articular tissues, 1 was confined in the TMJ space and 3 grew only in peri-articular soft and hard tissues, strictly adherent to the capsule but with no evidence of involvement of the TMJ space and structures. 2 cases of localized giant cell tumour of the synovium, the above mentioned localized nodular synovitis, were detected and thus identified as GCTTS, according to WHO nomenclature ( Table 1 ). No case of extra-articular GCTTS was found, which is peculiar to the synovium of tendons, and is absent in the TMJ.

Table 1

Clinical features and pattern of involvement of the temporomandibular joint by giant cell tumour reported in the English-language literature. ^*

Case	Author	Age/sex	Clinical features	Pre-operative diagnosis	Bony destruction	Treatment	Recurrence/months
Diffuse type with both extra-articular and intra-articular involvement
1–2	Lapayowker et al. (1973)	22/M	Preauricular mass, pain	Parotid tumour	Yes	SE, partial capsulectomy	NR
		58/F	Preauricular mass, decreased hearing, trismus	NR	Yes	NR	NR
3	Barnard et al. (1975)	37/M	Preauricular mass, pain, trismus	PVNS	Yes	SE	NR
4	Dinerman et al. (1977)	58/F	Preauricular mass, decreased hearing, tinnitus, clicking	PVNS	Yes	SE, dura mater excision	No/48
5	Miyamoto et al. (1977)	34/M	Preauricular mass, trismus	Parotid tumour	No	SE	No/24
6	Raibley (1977)	62/F	Preauricular mass, pain	Malignant parotid tumour	Yes	SE	No/5
7	Tagagi and Ishikawa (1981)	36/M	Preauricular mass, sub-trismus, clicking	NR	Yes	SE, condilectomy	Yes/60
8	Gallia et al. (1982)	47/F	Preauricular mass, sub-trismus, pain	NR	Yes	SE	No/132
9	Rickert and Shapiro (1982)	39/F	Parotid mass	PVNS	NR	SE, partial capsulectomy	NR
10	Curtin et al. (1983)	47/F	Preauricular mass, pain, sub-trismus	PVNS	Yes	SE	NR
11	O’Sullivan et al. (1984)	61/F	Preauricular mass	Parotid tumour	Yes	SE, radiotherapy	Yes/36
12	Dawiskiba et al. (1989)	32/M	Preauricular mass, trismus, pain	PVNS (biopsy)	No	NR	NR
13	Eisig et al. (1992)	50/F	Hearing loss	Central giant cell granuloma	Yes	SE, condilectomy	No/12
14	Syed et al. (1993)	10/F	Preauricular mass	Parotid tumour	No	SE, partial capsulectomy	NR
15	Franchi et al. (1994)	59/F	Preauricular mass, pain	Parotid tumour	No	SE, partial capsulectomy	No/12
16	Shapiro et al. (1996)	36/M	Preauricular mass, pain, hearing loss, clicking	NR	Yes	SE	No/18
17	Youssef et al. (1997)	41/F	Preauricular mass, pain	PVNS	Yes	SE, synoviectomy	No/12
18	Rubin et al. (1997)	70/F	Preauricular mass, sub-trismus, clicking	NR	Yes	SE, synoviectomy	No/36
19	Allis-Montmayeur (1997)	39/F	External ear canal mass	NR	NR	NR	No/7
20	Tanaka et al. (1997)	47/M	Preauricular mass, sub-trismus, pain	Giant cell granuloma	Yes	SE, condilectomy, craniectomy	No/24
21	Yu et al. (1997)	48/M	Preauricular mass, pain	Parotid tumour	No	SE	NR
22	Chow et al. (1998)	42/F	Zygomatic mass	Giant cell tumour, chondroblastoma	Yes	SE, condylectomy, capsulectomy	No/24
23	Omura et al. (1998)	18/M	Preauricular mass, sub-trismus, pain	NR	Yes	SE	No/24
24	Bemporad et al. (1999)	37/M	Preauricular mass, sub-trismus, hearing loss	PVNS	Yes	SE	NR
25	Song et al. (1999)	57/F	Preauricular mass	Parotid tumour	Yes	SE, condilectomy	No/24
26	Lee et al. (2000)	59/F	Preauricular mass	PVNS	Yes	SE, condilectomy	No/24
27	Klenoff et al. (2001)	35/NR	Preauricular mass, sub-trismus, pain, tinnitus	Parotid tumour	Yes	SE, mandibulectomy, craniectomy	No/36
28	Shapiro et al. (2002)	36/M	Temporal/preauricular mass	PVNS (biopsy)	Yes	SE, craniectomy	No/84
29–30	Church et al. (2003)	42/M	Preauricular mass, clicking, hearing loss	PVNS (biopsy)	Yes	SE, partial mandibulectomy	No/36
		33/M	Preauricular mass, sub-trismus, pain	PVNS (biopsy)	Yes	SE, partial mandibulectomy	No/24
31	Heo et al. (2003)	45/M	Preauricular mass, sub-trismus, clicking, tinnitus	PVNS	Yes	NR	NR
32	Aoyama et al. (2004)	33/M	Preauricular mass, sub-trismus, clicking	Giant cell tumour, malignant tumour	Yes	SE, condilectomy	No/24
33–36	Kim et al. (2004)	28/F	NR	NR	Yes	NR	NR
		22/M	NR	NR	Yes	NR	NR
		47/M	NR	NR	Yes	NR	NR
		58/F	NR	NR	Yes	NR	NR
Diffuse type with extra-articular and intra-articular involvement
37	Tosun et al. (2004)	60/M	Zygomatic mass, pain, trismus	PVNS (biopsy)	Yes	SE, condilectomy	No/84
38–39	Stryjakowska et al. (2005)	36/M	Preauricular mass, sub-trismus, tinnitus	PVNS (biopsy)	Yes	SE, mandibulectomy	No/36
		36/F	Preauricular mass, pain	NR	Yes	SE, partial capsulectomy	Yes/12
40–41	Oda et al. (2007)	52/M	Tightness in ear	NR	Yes	SE	No/11
		67/M	Cheek mass, pain	NR	Yes	SE	No/87
42	Chen et al. (2007)	52/M	Preauricular mass, sub-trismus, pain	NR	Yes	SE, condilectomy	NR
43	Cascone et al. (2008)	78/M	Preauricular mass	NR	No	SE, partial synoviectomy	No/24
44	Chen et al. (2008)	56/F	Hearing loss, temporal pain	NR	Yes	SE, craniectomy, radiotherapy	No/9
45	Day et al. (2008)	38/M	Zygomatic mass, pain	Cholesterol cyst, aneurismal bone cyst	Yes	SE, partial synoviectomy	No/3
46	Herman et al. (2009)	36/M	Preauricular mass, sub-trismus, pain	NR	Yes	SE, craniectomy	No/132
47	Shkoukani et al. (2009)	74/F	Preauricular mass, sub-trismus, pain	NR	Yes	SE, condilectomy	No/NR
48	Leiggener et al. (2010)	22/F	Pain, trismus	NR	Yes	SE, condilectomy	No/36
49	Lu et al. (2010)	42/M	Preauricular mass	PVNS (biopsy)	Yes	SE, synoviectomy	Yes/24
50	Romaňach et al. (2011)	26/M	Preauricular mass, trismus, pain, hearing loss	Osteosarcoma, chondrosarcoma, metastasis	Yes	SE, synoviectomy, craniectomy, radiotherapy	No/18
Diffuse type with only intra-articular involvement
51	Kişnişci et al. (2001)	45/F	Preauricular mass, sub-trismus, pain	NR	Yes	SE, partial capsulectomy	NR
Diffuse type with only extra-articular involvement
52	Cascone et al. (2005)	38/F	Preauricular mass	NR	No	SE, parotidectomy	No/12
53	Gong et al. (2010)	34/M	Preauricular mass, trismus	Giant cell containing lesion (biopsy)	Yes	SE, craniectomy	No/24
54	Present case	63/M	Preauricular mass	Parotid tumour	No	SE, partial parotidectomy	No/11
Localized type with only intra-articular involvement
55	Makek et al. (1978)	55/F	Preauricular mass, trismus, pain	NR	Yes	SE, capsulectomy	No/9
56	Cai et al. (2009)	21/F	Preauricular mass, sub-trismus, pain, clicking	NR	No	SE, synoviectomy	No/13

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