Diffuse-Type Tenosynovial Giant Cell Tumor Arising in the Temporomandibular Joint Extending to the External Auditory Canal: A Case Report and Literature Review

Abstract

A 74-year-old Japanese woman with a 1-year history of right preauricular pain and a 2-month history of bleeding from the right ear was admitted to our department. Tumor was observed in the anterior wall in the right external auditory canal. Bony swelling of the right preauricular area was palpated. Computed tomography revealed an ill-defined, osteogenic tumor around the mandibular condyle with a destructive bony lesion involving the temporal bone. Magnetic resonance imaging revealed a 2.0 × 1.5 × 1.3-cm solid tumor around the mandibular condyle, exhibiting a low-intensity signal on T1-weighted imaging and an isointense central area surrounded by low-signal intensity on T2-weighted imaging. Histological examination of biopsy specimens revealed diffuse-type tenosynovial giant cell tumor (D-TGCT). After the feeding arteries for the tumor were embolized, the patient underwent surgery with combined temporal craniotomy and mandibular condylectomy. The soft and cystic tumor with calcification located in the extradural space was totally resected along with the mandibular condyle. No facial paralysis or recurrence was evident as of 6 months postoperatively. To date, only 23 cases of D-TGCT arising in the temporomandibular joint (TMJ) with ear involvement have been reported since 2011. We report successful resection of a rare case of D-TGCT arising in the TMJ.

Introduction

Diffuse-type tenosynovial giant cell tumor (D-TGCT), also known as pigmented villonodular synovitis, is a rare, benign neoplastic neoplasm that arises from the synovial membrane, tendon sheaths, or bursae of large joints such as the hips, knees, or shoulder. ¹ The tumor often shows locally aggressive behavior and has the potential to invade into adjacent structures. D-TGCT arising in the temporomandibular joint (TMJ) is relatively rare, with approximately 100 cases reported.^2-4 D-TGCT arising in the TMJ usually invades to the temporal and mandibular bones, but D-TGCT extending to the ear is rare, with only 23 cases reported since 2011.^2,3,5-15 We report herein the successful resection of a case of D-TGCT arising in the TMJ presenting with bleeding from the external auditory canal (EAC).

Case Report

A 74-year-old Japanese woman with a 1-year history of right preauricular pain and a 2-month history of bleeding from the right ear was admitted to our department. A granulomatous, easily bleeding tumor was observed in the anterior wall of the right EAC (Figure 1A). Bony swelling of the right preauricular area was palpated. The patient reported no hearing loss, tinnitus, or trismus. The right ear drum was intact. Laboratory examination and hearing test showed normal results. Computed tomography (CT) revealed an ill-defined, osteogenic tumor around the mandibular condyle with a destructive bony lesion involving the temporal bone (Figure 1B). The tumor extended to the EAC, but not to the middle ear (Figure 1C). Axial section magnetic resonance imaging (MRI) revealed a 2.0 × 1.5 × 1.3-cm solid tumor around the mandibular condyle, exhibiting a low-intensity signal on T1-weighted imaging (Figure 1D) and an isointense central area surrounded by low-signal intensity on T2-weighted imaging (Figure 1E). Coronal section MRI revealed a signal hypointense tumor around the mandibular condyle on both T1- and T2-weighted images (Figure 1F and G).

Figure 1.

Tumor is observed in the anterior wall of the right external auditory canal (A). Computed tomography (CT) reveals an ill-defined osteogenic tumor around the mandibular condyle, with a destructive bony lesion involving the temporal bone. The tumor extends to the external auditory canal (B), but not to the middle ear (C). Axial section magnetic resonance imaging (MRI) reveals a 2.0 × 1.5 × 1.3-cm solid tumor around the mandibular condyle, exhibiting a low-intensity signal on T1-weighted imaging (D) and an isointense central area surrounded by low-signal intensity on T2-weighted imaging (E). Coronal section MRI reveals a low-intensity tumor around the mandibular condyle on both T1- (F) and T2-weighted imaging (G). Tumor is indicated by triangle.

We obtained biopsies from the tumor in the right EAC and preauricular area by skin incision. Histologically, hematoxylin and eosin staining demonstrated an admixture of small histocyte-like cells with cleaved nuclei, larger epithelioid cells with hemosiderin granules, and osteoclast-like multinuclear giant cells (Figure 2A). Tumor cells were CD68-positive (Figure 2B) and Ki-67 index was 30% (Figure 2C). Negative results were obtained for CD1a, CD34, and S-100. Histological examination revealed D-TGCT.

Figure 2.

Hematoxylin and eosin staining demonstrate an admixture of small, histocyte-like cells with cleaved nuclei, larger epithelioid cells with hemosiderin granules, and osteoclast-like multinuclear giant cells (A). Tumor cells are CD68-positive (B) and Ki-67 index is 30% (C). Scale bar: 50 μm.

Angiographic examination by catheterization from the right femoral artery to the right external carotid artery using Seldinger’s technique suggested that the feeding arteries for the tumor were the middle meningeal, superficial temporal, and internal maxillary arteries. These arteries were embolized using Embosphere (Merit Medical) containing 300 to 500 µm of particles. The next day, the patient underwent surgery with combined temporal craniotomy and mandibular condylectomy under nasotracheal intubation general anesthesia. A skin incision was made from the right earlobe via preauricular area to the temporal area (Figure 3A). After exposing the parotid gland, the main trunk and the branches of the facial nerve were preserved using a nerve-monitoring system (NIM Response 3.0, Medtronic Xomed; Figure 3B). The mandibular condyle and the lateral pterygoid muscle were cut below the facial nerve, then lower edge of the tumor was exposed. After the temporal muscle was reflected, a part of temporal squama, the posterior root of the zygomatic arch, and the superior mastoid air cells were resected by neurosurgeons. The soft and cystic tumor with calcification was located in the extradural space and separated from the dura mater of the middle cranial fossa (Figure 3C). After the location of the roof of the epitympanum was confirmed using the navigation system (Stealth Station S7; Medtronic). The tumor was totally resected along with the mandibular condyle (Figure 3D). The dura mater and perforated EAC were covered with temporalis fascia and the dead space filled with abdominal adipose tissue. Total bleeding volume was 130 mL and operative time was 5 hours. The postoperative clinical course was uneventful. The patient was discharged 12 days after surgery. No facial paralysis or recurrence have been found as of 6 months postoperatively. Slightly deviation of the jaw to the right was observed when opening the mouth as of 6 months.

Figure 3.

A, A skin incision is made from the right earlobe via the preauricular area to the temporal area. B, After exposing the parotid gland, the main trunk and the branches of the facial nerve are preserved, then the mandibular condyle and the lateral pterygoid muscle are cut below the facial nerve. C, After reflecting the temporal muscle, temporal craniotomy is performed. The soft and cystic tumor with calcification was located in the extradural space and separated from the dura mater of the middle cranial fossa. The tumor is totally resected along with the mandibular condyle. D, Appearance after the resection. D indicates dura mater; EAC, external auditory canal; FN, facial nerve; MC, mandibular condyle; P, parotid gland; T, tumor.

Discussion

The EAC is located behind the TMJ. D-TGCT arising in the TMJ thus often extends to the ear including EAC, and the middle and inner ear. To date, clinical characteristics and influences by the extension to the ear of D-TGCT arising in the TMJ have not been fully elucidated. We searched for the cases of D-TGCT arising in the TMJ extending to the ear in the literature since 2011. To our knowledge, only 23 cases were identified, and these cases are summarized in Table 1.^2,3,5-15 The median age of these patients was 53 years (range, 19-80 years). In terms of gender ratio, this pathology appears more frequent among males (17 males, 6 females). A variety of ear symptoms have been described in those 23 patients including hearing loss in 16 (70%) patients, pain in the ear or TMJ in 8 (35%), aural fulness in 7 (30%), EAC tumor or swelling in 6 (26%), otorrhea including ear bleeding in 4 (17%), otitis media in 3 (13%), dizziness in 3 (13%), and tinnitus in 3 (13%). Symptoms excluding the ear have included swelling or mass in the temporal or preauricular region in 8 of the 23 patients (35%), trismus in 5 (21%), headache in 2 (9%), and facial numbness in 2 (9%). The median period from symptom onset to consultation was 24 months (range, 1-60 months). According to imaging modalities such as CT and MRI and clinical findings, tumor extended to the EAC in 100% (19/19 cases), the middle ear in 70% (16/23), and the inner ear in 9% (2/22), and invaded to the dura mater in 24% (5/21). CT usually shows a tumorous lesion accompanied by bone destruction, ossification, and cystic change in the TMJ, mandibular condyle, and temporal bone. ¹³ Confirming the anatomical structures except for bones on CT such as pterygoid muscles, parotid gland, inner and middle ears, mastoid, carotid artery, and temporal lobe is important to determine the optimal route for surgical access. ¹⁰ Low-intensity signals from D-TGCT are frequently seen on T2-weighted MRI. This is attributed to hemosiderin deposition, known as the “blooming effect” as seen in the present case. ²

Table 1.

Clinical Characteristics of Reported Cases of Diffuse-Type Tenosynovial Giant Cell Tumor of the Temporomandibular Joint With Ear Involvement Since 2011.

No.	Age/gender	Symptoms and findings		Months from symptom onset to consultation	Side	Size (mm)	Extension			Dura invasion	Treatment	Complication	Follow-up (month)	Recurrence	Author
		Ear	Other				External auditory canal	Middle ear	Inner ear
1	59/Female	ET, HL, P	S	60	Left	38 × 35 × 35	+	–	–	–	Surgery	–	10	–	Cai(2011)[5]
2	80/Male	ET, HL, P	He, Tr	2	Left	NR	+	–	–	–	Surgery	–	18	–	Aimoni et al 6
3	56/Female	D, HL, OM, P	Tr	5	Left	34	NR	+	–	–	Surgery RT(35 Gy)	FP	28	–	Liu et al 4
4	52/Male	P	He, S	1	Right	52 × 27	+	+	–	NR	Surgery RT (45 Gy)	–	6	–	Safaee et al 8
5	30/Male	Ot	S	36	Left	25 × 20 × 15	+	–	–	–	Surgery	NR	NR	NR	Qin et al 9
6	40/Male	HL		NR	Right	NR	+	+	+	+	Surgery RT (60 Gy)	HL	240	–	Carlson et al 10
7	58/Male	AF, HL		24	Left	NR	+	+	–	–	Surgery RT (60 Gy)	NR	226	–
8	60/Male	AF, HL, P		3	Left	NR	+	+	–	–	Surgery	NR	162	–
9	57/Male	AF, HL, Ti		12	Left	NR	+	+	–	–	Surgery	NR	156	–
10	49/Male	P	Tr	24	Left	NR	+	+	–	–	Surgery	NR	51	+
11	79/Male	AF, HL, Ot		24	Right	NR	+	+	–	–	Surgery	NR	29	–
12	54/Male	HL, Ot		36	Right	NR	+	+	–	+	Surgery	NR	53	–
13	65/Female	ET, D, HL	FN	10	Right	NR	+	+	+	–	Surgery	FP	24	–	Vaca et al 11
14	54/Male	HL	S, Tr	36	Right	50	NR	+	NR	+	Surgery RT (45 Gy)	HL	10	+	Verspoor et al 2
15	52/Male	D, HL, Ti	S	24	Left	58 × 38 × 46	NR	+	–	+	Surgery RT	–	24	–	Yan et al 12
16	50/Male	HL, Ti		12	Left	53 × 36 × 33	NR	+	–	NR	Surgery RT	–	12	–
17	19/Female		S	24	Left	NR	+	+	–	–	Surgery	NR	12	–	Wang et al 3
18	40/Female	AF, HL, OM, P	FN, Tr	24	Right	56 × 45 × 46	+	+	–	–	Surgery	FP	30	+	Nomura et al 13
19	60/Male		S	48	Right	45 × 38 × 22	+	–	–	–	Surgery	FP	25	–
20	60/Male	ET		7	Right	25 × 28 × 26	+	–	–	–	Surgery	FP	4	–
21	46/Male	AF, HL, OM		3	Right	50	+	+	–	+	Surgery	He, FBS	16	–	Brant et al 14
22	42/Male	AF, ET, HL		2	Left	NR	+	–	–	–	Surgery	–	6	–	Vassalli et al 15
23	74/Female	ET, Ot, P	S	12	Right	20 × 15 × 13	+	–	–	–	Surgery	De	6	–	Present case

Abbreviations: AF, aural fullness; D, dizziness; De, deviation of jaw; ET, external auditory canal tumor or swelling; FBS, first bite syndrome; FN, facial numbness; FP, facial palsy; He, headache; HL, hearing loss; NR, not reported, OM, otitis media; Ot, otorrhea including ear bleeding; P, pain in the ear or temporomandibular joint; RT, radiotherapy; S, swelling or mass in the temporal region; Ti, tinnitis; Tr, trismus; –, absent; +, present.

According to the World Health Organization classification of bone and soft tissue tumors, D-TGCT is diagnosed from the histological findings as an admixture of small histocyte-like cells with cleaved nuclei, larger epithelioid cells with hemosiderin granules, and osteoclast-like multinuclear giant cells. ¹ In immunostaining, tumor cells stain positively for CD68, a marker of histocyte-like cells and mononuclear cells. Differential diagnoses of tumors around the TMJ include synovial chondromatosis, parotid gland carcinoma, and malignant bone and soft tissue tumors. ¹⁰ In terms of granulomatous tumors in the EAC, various neoplastic or infectious diseases can be considered, including carcinoma, tumor of the ceruminous glands, malignant external otitis, cholesteatoma, and cholesterin granuloma. ¹⁶

Preoperative embolization was reportedly useful for facilitating complete resection by limiting intraoperative hemorrhage and diminishes the risk of morbidity by improving visualization of important normal anatomic structures within the surgical field, ¹⁷ since D-TGCT of the TMJ is a highly vascular tumor. ¹⁸ Surgical resection is performed for all cases in Table 1. Complications of surgery have been reported as facial paralysis, hearing loss, and limited jaw movement. In the present case, we totally resected the tumor by surgery with combined temporal craniotomy and mandibular condylectomy. The intraoperative navigational system facilitated a correct understanding of relevant anatomical features. As the location of the roof of the epitympanum was confirmed using the navigation system, we were able to avoid damaging the middle ear, allowing preservation of hearing. In addition, we repeatedly monitored the integrity of the facial nerve using the nerve monitoring system. These initiatives contributed to the safe, curative resection without any major postoperative complications.

In previous cases, postoperative radiotherapy was combined with surgery in 7 (30%) of the 23 patients (Table 1). Postoperative radiotherapy (35-45 Gy) was applied for cases showing unclear margins or residual D-TGCT arising in the TMJ. ³ D-TGCT has been reported to show low sensitivity to radiotherapy. ¹⁰ Furthermore, radiotherapy can cause adverse effects such as dermatitis, nausea, alopecia, otitis media, and hearing loss. ² In the present case, the tumor was totally resected, so postoperative radiotherapy was not performed. Median duration of follow-up for previous cases was 24 months (range, 6-240 months; Table 1). Recurrence was observed in 3 (14%) of the 22 patients for which follow-up was reported, higher than in D-TGCT of the major joints treated with surgery, presumably due to the tendency toward a higher frequency of incomplete resection for D-TGCT of the TMJ. ⁸ Therefore, a long-term clinical and radiographical follow-up is necessary.

Conclusion

We reported successful resection of a case of D-TGCT arising in the TMJ without major postoperative complications. In patients with tumor in the EAC and preauricular swelling, clinicians should be aware of the possibility of D-TGCT of the TMJ.