Case Report
Chondrosarcoma of the temporomandibular joint: a case report and review of the literature Kyu-Young Oh, Hye-Jung Yoon, Jae-Il Lee, Sam-Pyo Hong, Seong-Doo Hong Department of Oral Pathology, School of Dentistry and Dental Research Institute, Seoul National University, Republic of Korea Objective: Chondrosarcoma is the second most common sarcoma arising in the bone, but it rarely involves the temporomandibular joint (TMJ). To date, 30 cases of TMJ chondrosarcoma have been reported in the English literature, and the authors report an additional case arising from a cystic lesion in a 60-year-old female patient. Clinical presentation: The clinical and radiological diagnosis of the lesion was initially synovial cyst, and periodic check-ups were done after aspiration of the lesion. After 3 years, the patient perceived swelling of the lesion, and surgical excision was performed. The final diagnosis was Grade I chondrosarcoma, and further immunohistochemical examination was carried out with S-100 protein, D2-40, CD68, and Ki-67. Conclusion: When clinicians detect a cystic lesion in the radiographic imaging of the TMJ, chondrosarcoma should be included in the differential diagnosis. In addition, computed tomography (CT) as well as magnetic resonance imaging (MRI) is recommended for the accurate diagnosis and proper preoperative planning in TMJ chondrosarcoma. Lastly, further studies on immunohistochemical examination would be helpful for differential diagnosis in the case of small biopsy in the TMJ. Keywords: Chondrosarcoma, Temporomandibular joint, TMJ, Immunohistochemistry, Secondary chondrosarcoma
Introduction Chondrosarcoma is a malignant tumor composed entirely of a hyaline cartilage matrix and chondrocytes in lacunae without osteoid formation.1,2 Chondrosarcoma constitutes approximately 11% of all primary malignant bone tumors and is the second most common sarcoma arising in the bone, following osteosarcoma.1 More than two-thirds of chondrosarcoma cases involve the pelvis, shoulder girdles, and the upper ends of the femur and humerus.2 Chondrosarcoma in the head and neck has been reported to be rare, ranging from 1 to 12% of all cases of chondrosarcoma.1 In the head and neck, the most common sites of occurrence are the larynx, followed by the mandible, maxilla, and maxillofacial skeleton.3 This report presents a case of chondrosarcoma in the temporomandibular joint (TMJ) arising from a cystic lesion. In TMJ cases, there were three reports of malignant transformation of benign lesions into chondrosarcoma,4–6 but a cystic lesion such as a synovial cyst has not been reported to be a preexisting Correspondence to: Seong-Doo Hong, Department of Oral Pathology, School of Dentistry, Seoul National University, Daehak-ro 101, Jongno-gu, Seoul, Republic of Korea. Email: [email protected] ß W. S. Maney & Son Ltd 2015 DOI 10.1179/2151090315Y.0000000016
benign lesion. In addition, only a few immunohistochemical studies have been carried out in TMJ chondrosarcoma, so the results of additional immunohistochemical examination will be dealt with. To date, 30 cases of TMJ chondrosarcoma have been reported in the English literature, and they will be summarized and discussed in this review.
Case Report A 60-year-old woman presented with limited mouth opening due to pain in the right TMJ. Magnetic resonance imaging (MRI) showed a cystic lesion in the right infratemporal fossa. The clinical diagnosis was synovial cyst in the right TMJ. An aspiration was performed through the sigmoid notch, and the aspirated fluid was suggestive of synovial fluid. Two months later, the patient presented suddenly, complaining of pain and paresthesia of the right TMJ. An MRI scan was performed again and revealed cystic dilatation with mild synovial enhancement (Fig. 1). Considering these radiological features, the differential diagnosis included synovial cyst and pigmented villonodular synovitis, which was less likely. The patient refused to undergo surgery, so periodic check-ups were carried out.
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Figure 1 Magnetic resonance imaging (MRI) taken 2 months after the first visit. (A) Axial gadolinium-enhanced T1-weighted MRI showed that the right condyle was surrounded by a cystic lesion with mild synovial enhancement. (B) Axial T2-weighted MRI showed high signal intensity.
At her 3-year check-up, the patient perceived swelling of the right TMJ. On the third MRI, there was an increase in size of the cystic lesion, with the greatest diameter being 5.3 cm, but destruction of the condyle was not conspicuous (Fig. 2). A punch biopsy was taken through the masticator space, and the result was a hyperplastic cartilage chip, with the recommendation for excision of the entire mass. A computed tomography (CT) taken after the punch biopsy showed a lobulated and low attenuated mass surrounding the right condyle. The mass had a peripheral enhancing rim and internal septa, and periosteal reaction was detected. Destruction of the right condyle and erosion of the adjacent cranial base were also found (Fig. 3).
Surgical excision was performed with partial mandibulectomy, including the condyle and coronoid process, after an osteotomy of the right zygomatic arch for surgical approach. During the surgical procedure, involvement of the articular fossa was confirmed by frozen-section examination, and further resection was done, including the adjacent dura mater. Subsequently, intermaxillary fixation was done, and the separated zygomatic arch was repositioned and fixed with a metal plate and screws. For reconstruction, an artificial fossa prosthesis made of ultra-high molecular weight polyethylene was fixed on the glenoid fossa, and a plate with an artificial condyle head was fixed into the mandibular angle. In addition, the latissimus dorsal flap was used for soft tissue reconstruction.
Figure 2 MRI taken 3 years after the first visit. (A) Axial gadolinium-enhanced T1-weighted MR imaging. A remarkable increase in size of the cystic lesion was found compared with Fig. 1A. Most of the masticator, retroantral, and parotid spaces were filled with the lesion. (B) Axial T2-weighted MR imaging. Most portions of the lesion showed high signal intensity.
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Figure 3 Computed tomography (CT) taken after punch biopsy. (A) Axial and (B) coronal CT scans with bone setting presented prominent condylar resorption (arrows) and erosion of the adjacent cranial base (arrow head). (C) Axial and (D) coronal CT scans with soft tissue setting revealed an enhancing peripheral rim (white arrows) and internal septa (black arrows).
Histopathologic examination of the excised mass primarily showed lacunar formation within the chondroid matrix (Fig. 4A). Chondrocytes in the lacunae presented with variation in size and shape, a few of which were double nucleated (Fig. 4B). Occasionally, two chondrocytes occupied one lacuna. Mitotic figures were extremely rare. The lesion grew in lobules of various sizes, some of which coalesced, and the lobules were separated by thin fibrous tissue septa (Fig. 4C). Neither necrosis nor calcification was found within the chondroid matrix. Permeation into the medullary bone of the articular fossa was detected (Fig. 4D). In addition, immunohistochemical examination was carried out with antibodies against S-100 protein (Clone 4C4.9, 1:300, Cell marque), D2-40 (Clone D2-40, 1:200, Dako), CD68 (Clone PG-M1, 1:100, Dako), and Ki-67 (Clone MIB-1, 1:400, Dako). S-100 was strongly expressed in 80% of the chondrocytes, whereas D2-40 was weakly expressed in 90%. CD68 and
Ki-67 were positive in 5% and v1%, respectively. The final diagnosis was Grade I chondrosarcoma. Because involvement of the superior margin was confirmed in the histopathologic examination, the patient received 54 Gy radiotherapy in 30 fractions. There was no evidence of recurrence or distant metastasis on CT and positron emission tomography (PET) scans at an 8-month follow-up visit (Fig. 5).
Discussion From 1954 to the present, 30 cases of TMJ chondrosarcoma have been reported in the English literature (Table 1).3–29 The age ranged from 7 to 75 years old, with the mean age of 46.5 years. The mean age of chondrosarcoma in TMJ was slightly higher than that in the head and neck.30,31 A female predominance, with a female-to-male ratio of 1.8:1, was found, which was not consistent with a male predominance in the head and neck or other body parts.30–32
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Figure 4 Histopathologic features of temporomandibular joint (TMJ) chondrosarcoma. Hematoxylin and eosin stains. (A) Lobular growth pattern of numerous lacunae within a chondroid matrix ( 3 100). (B) Chondrocytes showed marked pleomorphism, and a double-nucleated chondrocyte was found (arrow, 3 400). (C) Fibrous tissue septa separating coalesced lobules ( 3 200). (D) Tumor invasion into the medullary bone ( 3 200).
Figure 5 Panoramic radiograph at an 8-month postoperative follow-up.
Clinically, swelling was the most frequent symptom of TMJ chondrosarcoma, followed by pain, trismus, and hearing loss (Fig. 6). Headache, facial asymmetry, and paresthesia were each reported once. The major radiological features of TMJ chondrosarcoma were condylar resorption, erosion of adjacent bone, and calcification within the mass (Fig. 7). In three reported cases, more destructive bony change than erosion was detected in the external auditory canal, petrous temporal bone, and mandibular ramus.3,19,27 An enhancing peripheral rim was found in four cases and a periosteal reaction in two cases. Oliveira et al.22 mentioned the importance of MRI in the differential diagnosis of TMJ 4
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chondrosarcoma. In their case report, hypercellular content of the lesion and the presence of a chondroid or myxoid matrix were confirmed through MRI. In addition, MRI was recommended for preoperative planning because it provided detailed information on the anatomic limits and the most accurate view of the magnitude of the lesion.22,24 In the present case, however, condylar destruction, erosion of the cranial base, and mass architecture, including an enhancing peripheral rim and internal septa, were more distinctly found on CT than MRI. Given that these radiological findings could be clear bases suggesting chondrosarcoma rather than synovial cyst, CT as well as MRI is recommended for the accurate diagnosis and proper preoperative planning in TMJ chondrosarcoma. The time from the first perception of symptoms to the initial visit to the clinic ranged from 2 to 96 months in 26 cases. The mean time of 20.7 months in TMJ chondrosarcoma, approximately three times longer than that in head and neck chondrosarcoma,31 suggested the propensity for slow growth of the lesion, allowing for its malignancy, and should be considered by clinicians so as not to miss chondrosarcoma in the differential diagnosis. In 22 reported cases, the greatest diameter of TMJ VOL .
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Tullio (1974)9 Nortje´ (1976)10
Sato (1977)11
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17
18
Bernasconi (2004)20 Gallego (2009)21 Oliveira (2009)22 Garzino-Demo (2010)23
Mostafapour (2000)3
2015
VOL .
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65
21
22
54
65
16
20
66
15
55
60
14
19
50
13
00
F
F
M
F
F
F
M
F
F
F
F
36
Merrill (1997)16b Sesenna (1997)17 Ichikawa (1998)18b Batra (1999)19
F
F
29
49
F
–
– –
60
–
– –
F M
F M
F
11 Wasenko (1990)14 15 12 Nitzan (1993)
9 Cadenat (1979)12 Morris (1987)13 10
8
48 75
2 3
17 40
46
1
Gingrass (1954)7 Lanier (1971)4a Richter (1974)8
First author
3
2
3
48
18
96
18
12
12
36
72
–
24
–
4
36 18
8 6
24 10
12
Swelling, pain
Swelling, pain, trismus Pain, trismus
Pain, trismus
Swelling
Swelling, pain, trismus Swelling, hearing loss Swelling
Swelling
Swelling, pain, trismus Trismus
Swelling, headache Swelling, pain
Pain Swelling, pain, trismus Swelling, pain, trismus Swelling, pain
Swelling, pain, trismus Swelling, pain Swelling, pain, hearing loss Swelling Swelling, pain
Duration of Case symptoms No. Age Sex (months) Chief complaints
Mass with calcification, condylar resorption
Mass with enhancing peripheral rim, condylar resorption
Mass adjacent to condyle
Mass with enhancing peripheral rim, adjacent bony erosion
Adjacent bony destruction
Mass with calcification, adjacent bony erosion and destruction Condylar resorption
Mass with calcification
Mass with calcification
TMJ space widening, condylar resorption, adjacent bony erosion Radiopacity with radiolucency
Mass with calcification
Mass with calcification, adjacent bony erosion
–
Radiopacity with radiolucency
Condylar resorption TMJ space widening, elongated condyle, adjacent bony erosion Condylar resorption TMJ space widening, elongated condyle, radiopacity with radiolucency, adjacent bony erosion – Radiopacity with radiolucency
Bone deposition
Radiological features
size
I
I
2.7|1.9 –
I
2.2|1.3|0.5
I
6.0|4.0|3.0
I
I
6.0|6.0
3.5
I
–
I
–
2.2|1.5|0.7, 1.3|1.0|0.5 4.0|1.8
–
4.0
I
I
4.0|2.5 2.5
I
–
–
– –
– I
– –
–
Surgery, RT
Surgery
Surgery
Surgery, RT after recurrence Surgery
Surgery
Surgery
Surgery
Surgery
Surgery
Surgery
Surgery
Surgery, RT
Surgery
Surgery
Surgery, RT Surgery
Surgery Surgery
Surgery Surgery
Surgery
Treatment Grade surgery
2.5
–
–
– –
– 6.0
2.0 6.0|5.0|3.5
–
Tumor (cm)
Table 1 Thirty cases of temporomandibular joint (TMJ) chondrosarcoma reported in the English literature and the present case
9 years
3.5 years
16 months
1 year
12 months, recurrence
–
7 months
3 years
5 years
18 months
7 years
–
6 months
4 months
–
– –
– 24 months
14 months 12 months
–
Follow-up
Oh et al. Case report of TMJ chondrosarcoma
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5
6
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RamosMurguialday (2012)25 Abu-Serriah (2013)26 Goutzanis (2013)27 Coleman (2013)6d Giorgione (2013)28 Kumar Reddy (2014)29 Present case (2014)
Gonza´lezPe´rez (2011)24 Xu (2011)5c
First author
2015
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60
31
63
28
30
23
27
56
48
26
29
45
25
0
F
M
M
F
M
M
M
F
2
2
36
12
12
–
–
–
Swelling, pain, trismus, paresthesia
Swelling, pain, trismus Swelling, pain
Swelling
Swelling, pain
Pain
Trismus, facial asymmetry Asymptomatic
Lobulated mass with enhancing peripheral rim and internal septa, condylar resorption, adjacent bony erosion, periosteal reaction
Mass with calcification, condylar resorption, periosteal reaction Mass with calcification, condylar resorption
Lobulated chondroid mass, condylar resorption
Condylar resorption, adjacent bony destruction
Mass with calcification, adjacent bony erosion
Mass with calcification, condylar resorption, adjacent bony erosion Multilocular radiolucency with mandibular osteolysis
34
24
Swelling, pain
Mass with enhancing peripheral rim, condylar resorption
12
57
23
M
Radiological features
Duration of Case symptoms No. Age Sex (months) Chief complaints
Table 1 Continued
I
I
5.0|3.0 5.3
I
–
5.5|5.0|4.0 4.6|4.0|3.9
I
6.0
–
II
5.5|2.0 1.0
I
8.0|6.0|5.0
Surgery, RT
Surgery
Surgery, RT
Surgery, RT
Surgery
Surgery
Surgery
Surgery
Surgery
Treatment Grade surgery I
size
–
Tumor (cm)
5 months
1 year
–
–
2 years
6 months
3 years
–
2 years
Follow-up
Oh et al. Case report of TMJ chondrosarcoma
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Case report of TMJ chondrosarcoma
Figure 6 Chief complaints of 28 cases of TMJ chondrosarcoma in the English language literature.
Figure 7 Radiological features of 29 cases of TMJ chondrosarcoma in the English language literature.
chondrosarcoma ranged from 1.0 to 8.0 cm, with the mean size of 4.3 cm. The tumor size of chondrosarcoma in 2TMJ was similar to that in the skull base but smaller than that in the head and neck.31,33 Temporomandibular joint chondrosarcoma has typical histopathologic features of chondrosarcoma in that lacunae, including pleomorphic chondrocytes, show a lobular growth pattern within the chondroid matrix, and double-nucleated nuclei are occasionally detected. According to Evans et al.,32 chondrosarcoma has been classified into three grades on the basis of histologic criteria, including nuclear size, mitosis, cellularity, and matrix character. In this review on histologic grading of TMJ chondrosarcoma, by classifying ‘well-differentiated’ or ‘low grade’ into ‘Grade I’, 18 out of 19 cases were diagnosed as Grade I, with only one as Grade II. This demonstrated that the percentage of Grade I is much higher in TMJ chondrosarcoma than in other body parts.32,34 Malignant transformation from a benign lesion, including synovial chondromatosis, multiple chondroma, or osteochondroma, to chondrosarcoma has been reported as secondary chondrosarcoma.35 In previous studies, the malignant transformation rate in synovial chondromatosis was 5–10% and
that of solitary osteochondroma was 0.4–2%.35–37 In reports of TMJ chondrosarcoma, three cases of secondary chondrosarcoma arose from synovial chondromatosis, osteochondroma, and enchondroma, respectively.4–6 In the present case, the initial diagnosis was a synovial cyst, based on the MRI scan. Although the diagnosis was not definitive because a biopsy was not performed at that time, synovial cyst was the most probable diagnosis, based on the images showing the cystic lesion with synovial enhancement and the fluid from the aspiration. As treatment for a synovial cyst, an arthroscopic procedure instead of surgical excision was suggested for its conservativeness and management of inflammation.38 However, if the cyst does not disappear spontaneously after the arthroscopic procedure, surgical excision should be carried out to prevent aggravation of the lesion. Although malignant transformation of synovial cysts has not been reported to date, clinicians are advised to notice the possibility of malignant transformation, taking the present case into account. Out of 31 cases of TMJ chondrosarcoma, fine needle aspiration biopsy (FNAB) was performed in eight cases. Among these cases, the diagnosis of chondrosarcoma could be inferred from FNAB in
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only two cases; in one of these cases, the second FNAB was necessary for diagnosis due to inadequacy of the first biopsy.19,23 In four cases, FNAB was not indicative of chondrosarcoma20,24,27 or insufficient for definitive diagnosis.13 Two of the cases reported that the result of FNAB was pleomorphic adenoma.17,28 In the present case, a small punch biopsy was performed, and the result implied cartilage proliferation but was not sufficient for definitive diagnosis as chondrosarcoma. A small amount of tissue from FNAB or a punch biopsy in chondrosarcoma shows pleomorphic chondrocytes and double-nucleated nuclei, but these cellular features can also be seen in other chondroid lesions like synovial chondromatosis.39 To take advantage of FNAB in TMJ lesions suspicious for chondrosarcoma, immunohistochemical stains may play a major role in reaching a reliable diagnosis. To date, only six cases of TMJ chondrosarcoma had immunostaining.17,18,21,22,24,28 In the results, S-100 was positively expressed in 5–35% of chondrocytes17,21,24 and Ki-67 was in 5–50%.21,24,28 Compared to these results, the present case showed high expression of S-100 and low expression of Ki-67. Additionally, D2-40 and CD68 immunostains were carried out in the present case. D2-40 is a known marker of cartilaginous tumors and has been studied for its expression in chondrosarcoma.40–42 CD68 expression in chondroid lesions has been reported in two papers. One paper showed negative CD68 expression in clear cell chondrosarcoma as well as in conventional chondrosarcoma.43 The other paper reported positive expression in a few chondrocytes of primary synovial chondromatosis.44 In-depth studies of immunostains in chondrosarcoma compared to other chondroid lesions are necessary for accuracy in differential diagnosis, especially in cases of a small biopsy such as FNAB. Due to the absence of lymph node metastasis on preoperative imaging, all patients with TMJ chondrosarcoma were treated surgically without neck dissection. In 7 out of 31 TMJ chondrosarcoma cases, radiotherapy was performed after surgery and the mean dose was 63 Gy, averaged from doses reported in three cases, including the present case.13,23 Although chondrosarcoma had traditionally been regarded as a radioresistant tumor, Harwood et al.45 concluded that chondrosarcoma of the bone is a radioresponsive tumor. Burkey et al.46 insisted that postoperative radiotherapy can be useful in some extensive, high grade, or incompletely resected chondrosarcomas. Only one case of TMJ chondrosarcoma reported a recurrence 1 year after surgery.3 The patient in the 8
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case declined radiotherapy despite positive margins in the histopathologic analysis. Considering its efficacy as mentioned above, postoperative radiotherapy should be applied to cases of TMJ chondrosarcoma with incomplete surgical resection margin.
Disclaimer Statements Contributors K-YO, as a first author, contributed to selecting the case and writing the article in whole. H-JY, J-IL, and S-PH contributed in reviewing the literature and revising the article in part. S-DH, as a corresponding author, contributed in directing all the process and revising finally.
Funding Conflicts of interest There are no conflicts-of-interest.
Ethics approval Not required. Because this is a report of a single case not including private information such as patient’s identification and face photos.
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14 Wasenko JJ, Rosenbloom SA. Temporomandibular joint chondrosarcoma: CT demonstration. J Comput Assist Tomogr. 1990;14:1002–3. 15 Nitzan DW, Marmary Y, Hasson O, Elidan J. Chondrosarcoma arising in the temporomandibular joint: a case report and literature review. J Oral Maxillofac Surg. 1993;51:312–5. 16 Merrill RG, Yih WY, Shamloo J. Synovial chondrosarcoma of the temporomandibular joint: a case report. J Oral Maxillofac Surg. 1997;55:1312–6. 17 Sesenna E, Tullio A, Ferrari S. Chondrosarcoma of the temporomandibular joint: a case report and review of the literature. J Oral Maxillofac Surg. 1997;55:1348–52. 18 Ichikawa T, Miyauchi M, Nikai H, Yoshiga K. Synovial chondrosarcoma arising in the temporomandibular joint. J Oral Maxillofac Surg. 1998;56:890–4. 19 Batra PS, Estrem SA, Zitsch RP, McDonald R, Ditto J. Chondrosarcoma of the temporomandibular joint. Otolaryngol Head Neck Surg. 1999;120:951–4. 20 Bernasconi G, Preda L, Padula E, Baciliero U, Sammarchi L, Bellomi M. Parosteal chondrosarcoma, a very rare condition of the mandibular condyle. Clin Imaging. 2004;28:64–8. 21 Gallego L, Junquera L, Fresno MF, Carlos de Vicente J. Chondrosarcoma of the temporomandibular joint. A case report and review of the literature. Med Oral Patol Oral Cir Bucal. 2009;14:E39–E43. 22 Oliveira RC, Marques KDS, Mendonca AR, Mendoca EF. Chondrosarcoma of the temporomandibular joint: a case report in a child. J Orofac Pain. 2009;23:275–81. 23 Garzino-Demo P, Tanteri G, Boffano P, Ramieri G, Pacchiori D, Maletta F, et al. Chondrosarcoma of the temporomandibular joint: a case report and review of the literature. J Oral Maxillofac Surg. 2010;68:2005–11. 24 Gonza´lez-Pe´rez LM, Sa´nchez-Gallego F, Pe´rez-Ceballos JL, Lo´pez-Vaquero D. Temporomandibular joint chondrosarcoma: case report. J Cranio-Maxillofac Surg. 2011;30:79–83. 25 Ramos-Murguialday M, Lasa-Mene´ndez V, Iriarte-Ortabe JI, Couce M. Chondrosarcoma of the mandible involving angle, ramus, and condyle. J Craniofac Surg. 2012;23:1216–9. 26 Abu-Serriah M, Ahluwalia K, Shah KA, Bojanic S, Saeed N. A novel approach to chondrosarcoma of the glenoid fossa of the temporomandibular joint: a case report. J Oral Maxillofac Surg. 2013;71:208–13. 27 Goutzanis L, Kalfarentzos EF, Petsinis V, Papadogeorgakis N. Chondrosarcoma of the mandibular condyle in a patient with Werner syndrome: a case report. J Cranio-Maxillofac. 2013; 40:e170–4. 28 Giorgione C, Passali FM, Varakliotis T, Sibilia M, Ottaviani F. Temporo-mandibular joint chondrosarcoma: case report and review of the literature. Acta Otorhinolaryngol Ital. 2013;. 29 Kumar Reddy DS, Kishore Kumar RV, Gali R, Kannubaddy SR, Rao M, Akheel M. Central chondrosarcoma of a pediatric mandibular condyle: a case report and review. Ann Maxilofac Surg. 2014;4:85–9.
Case report of TMJ chondrosarcoma
30 Ruark DS, Schlehaider UK, Shah JP. Chondrosarcoma of the head and neck. World J Surg. 1992;16:1010–6. 31 Prado FO, Nishimoto IN, Perez DE, Kowalski LP, Lopes MA. Head and neck chondrosarcoma: analysis of 16 cases. Br J Oral Maxillofac Surg. 2009;47:555–7. 32 Evans HL, Ayala AG, Romsdahl MM. Prognostic factors in chondrosarcoma of bone. A clinicopathologic analysis with emphasis on histologic grading. Cancer. 1977; 40:818–31. 33 Meyers SP, Hirsch WL Jr, Curtin HD, Barnes L, Sekhar LN, Sen C. Chondrosarcomas of the skull base: MR imaging features. Radiology. 1992;184:103–8. 34 Bjo¨rnsson J, McLeod RA, Unni KK, Ilstrup DM, Pritchard DJ. Primary chondrosarcoma of long bones and limb girdles. Cancer. 1998;83:2105–19. 35 Ahmed AR, Tan TS, Unni KK, Collins MS, Wenger DE, Sim FH. Secondary chondrosarcoma in osteochondroma: report of 107 patients. Clin Orthop Relat Res. 2003;411:193–206. 36 Davis RI, Hamilton A, Biggart JD. Primary synovial chondromatosis: a clinicopathologic review and assessment of malignant potential. Hum Pathol. 1998;29:683–8. 37 Wittkop B, Davies AM, Mangham DC. Primary synovial chondromatosis and synovial chondrosarcoma. Eur Radiol. 2002;12:2112–9. 38 Bonacci CE, Lambert BJ, Pulse CL, Israel HA. Inflammatory synovial cyst of the temporomandibular joint: a case report and review of the literature. J Oral Maxillofac Surg. 1996;54: 769–73. 39 Sperling BL, Angel S, Stoneham G, Chow V, McFadden A, Chibbar R. Synovial chondromatosis and chondrosarcoma: a diagnostic dilemma. Sarcoma. 2003;7:69–73. 40 Huse JT, Pasha TL, Zhang PJ. D2-40 functions as an effective chondroid marker distinguishing true chondroid tumors from chordoma. Acta Neuropathol. 2007;113:87–94. 41 Daugaard S, Christensen LH, Høgdall E. Markers aiding the diagnosis of chondroid tumors: an immunohistochemical study including osteonectin, bcl-2, cox-2, actin, calponin, D2-40(podoplanin), mdm-2, CD117(c-kit), and YKL-40. APMIS. 2009;117:518–25. 42 Cho HY, Lee M, Takei H, Dancer J, Ro JY, Zhai QJ. Immunohistochemical comparison of chordoma with chondrosarcoma, myxopapillary ependymoma, and chordoid meningioma. Appl Immunohistochem Mol Morphol. 2009;17:131–8. 43 Masui F, Ushigome S, Fujii K. Clear cell chondrosarcoma: a pathological and immunohistochemical study. Histopathology. 1999;34:447–52. 44 Apte SS, Athanasou NA. An immunohistological study of cartilage and synovium in primary synovial chondromatosis. J Pathol. 1992;166:277–81. 45 Harwood AR, Krajbich JI, Fornasier VL. Radiotherapy of chondrosarcoma of bone. Cancer. 1980;45:2769–77. 46 Burkey BB, Hoffman HT, Baker SR, Thornton AF, McClatchey KD. Chondrosarcoma of the head and neck. Laryngoscope. 1990;100:1301–5.
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Chondrosarcoma of the temporomandibular joint: a case report and review of the literature Kyu-Young Oh, Hye-Jung Yoon, Jae-Il Lee, Sam-Pyo Hong, Seong-Doo Hong Department of Oral Pathology, School of Dentistry and Dental Research Institute, Seoul National University, Republic of Korea Objective: Chondrosarcoma is the second most common sarcoma arising in the bone, but it rarely involves the temporomandibular joint (TMJ). To date, 30 cases of TMJ chondrosarcoma have been reported in the English literature, and the authors report an additional case arising from a cystic lesion in a 60-year-old female patient. Clinical presentation: The clinical and radiological diagnosis of the lesion was initially synovial cyst, and periodic check-ups were done after aspiration of the lesion. After 3 years, the patient perceived swelling of the lesion, and surgical excision was performed. The final diagnosis was Grade I chondrosarcoma, and further immunohistochemical examination was carried out with S-100 protein, D2-40, CD68, and Ki-67. Conclusion: When clinicians detect a cystic lesion in the radiographic imaging of the TMJ, chondrosarcoma should be included in the differential diagnosis. In addition, computed tomography (CT) as well as magnetic resonance imaging (MRI) is recommended for the accurate diagnosis and proper preoperative planning in TMJ chondrosarcoma. Lastly, further studies on immunohistochemical examination would be helpful for differential diagnosis in the case of small biopsy in the TMJ. Keywords: Chondrosarcoma, Temporomandibular joint, TMJ, Immunohistochemistry, Secondary chondrosarcoma
Introduction Chondrosarcoma is a malignant tumor composed entirely of a hyaline cartilage matrix and chondrocytes in lacunae without osteoid formation.1,2 Chondrosarcoma constitutes approximately 11% of all primary malignant bone tumors and is the second most common sarcoma arising in the bone, following osteosarcoma.1 More than two-thirds of chondrosarcoma cases involve the pelvis, shoulder girdles, and the upper ends of the femur and humerus.2 Chondrosarcoma in the head and neck has been reported to be rare, ranging from 1 to 12% of all cases of chondrosarcoma.1 In the head and neck, the most common sites of occurrence are the larynx, followed by the mandible, maxilla, and maxillofacial skeleton.3 This report presents a case of chondrosarcoma in the temporomandibular joint (TMJ) arising from a cystic lesion. In TMJ cases, there were three reports of malignant transformation of benign lesions into chondrosarcoma,4–6 but a cystic lesion such as a synovial cyst has not been reported to be a preexisting Correspondence to: Seong-Doo Hong, Department of Oral Pathology, School of Dentistry, Seoul National University, Daehak-ro 101, Jongno-gu, Seoul, Republic of Korea. Email: [email protected] ß W. S. Maney & Son Ltd 2015 DOI 10.1179/2151090315Y.0000000016
benign lesion. In addition, only a few immunohistochemical studies have been carried out in TMJ chondrosarcoma, so the results of additional immunohistochemical examination will be dealt with. To date, 30 cases of TMJ chondrosarcoma have been reported in the English literature, and they will be summarized and discussed in this review.
Case Report A 60-year-old woman presented with limited mouth opening due to pain in the right TMJ. Magnetic resonance imaging (MRI) showed a cystic lesion in the right infratemporal fossa. The clinical diagnosis was synovial cyst in the right TMJ. An aspiration was performed through the sigmoid notch, and the aspirated fluid was suggestive of synovial fluid. Two months later, the patient presented suddenly, complaining of pain and paresthesia of the right TMJ. An MRI scan was performed again and revealed cystic dilatation with mild synovial enhancement (Fig. 1). Considering these radiological features, the differential diagnosis included synovial cyst and pigmented villonodular synovitis, which was less likely. The patient refused to undergo surgery, so periodic check-ups were carried out.
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Figure 1 Magnetic resonance imaging (MRI) taken 2 months after the first visit. (A) Axial gadolinium-enhanced T1-weighted MRI showed that the right condyle was surrounded by a cystic lesion with mild synovial enhancement. (B) Axial T2-weighted MRI showed high signal intensity.
At her 3-year check-up, the patient perceived swelling of the right TMJ. On the third MRI, there was an increase in size of the cystic lesion, with the greatest diameter being 5.3 cm, but destruction of the condyle was not conspicuous (Fig. 2). A punch biopsy was taken through the masticator space, and the result was a hyperplastic cartilage chip, with the recommendation for excision of the entire mass. A computed tomography (CT) taken after the punch biopsy showed a lobulated and low attenuated mass surrounding the right condyle. The mass had a peripheral enhancing rim and internal septa, and periosteal reaction was detected. Destruction of the right condyle and erosion of the adjacent cranial base were also found (Fig. 3).
Surgical excision was performed with partial mandibulectomy, including the condyle and coronoid process, after an osteotomy of the right zygomatic arch for surgical approach. During the surgical procedure, involvement of the articular fossa was confirmed by frozen-section examination, and further resection was done, including the adjacent dura mater. Subsequently, intermaxillary fixation was done, and the separated zygomatic arch was repositioned and fixed with a metal plate and screws. For reconstruction, an artificial fossa prosthesis made of ultra-high molecular weight polyethylene was fixed on the glenoid fossa, and a plate with an artificial condyle head was fixed into the mandibular angle. In addition, the latissimus dorsal flap was used for soft tissue reconstruction.
Figure 2 MRI taken 3 years after the first visit. (A) Axial gadolinium-enhanced T1-weighted MR imaging. A remarkable increase in size of the cystic lesion was found compared with Fig. 1A. Most of the masticator, retroantral, and parotid spaces were filled with the lesion. (B) Axial T2-weighted MR imaging. Most portions of the lesion showed high signal intensity.
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Figure 3 Computed tomography (CT) taken after punch biopsy. (A) Axial and (B) coronal CT scans with bone setting presented prominent condylar resorption (arrows) and erosion of the adjacent cranial base (arrow head). (C) Axial and (D) coronal CT scans with soft tissue setting revealed an enhancing peripheral rim (white arrows) and internal septa (black arrows).
Histopathologic examination of the excised mass primarily showed lacunar formation within the chondroid matrix (Fig. 4A). Chondrocytes in the lacunae presented with variation in size and shape, a few of which were double nucleated (Fig. 4B). Occasionally, two chondrocytes occupied one lacuna. Mitotic figures were extremely rare. The lesion grew in lobules of various sizes, some of which coalesced, and the lobules were separated by thin fibrous tissue septa (Fig. 4C). Neither necrosis nor calcification was found within the chondroid matrix. Permeation into the medullary bone of the articular fossa was detected (Fig. 4D). In addition, immunohistochemical examination was carried out with antibodies against S-100 protein (Clone 4C4.9, 1:300, Cell marque), D2-40 (Clone D2-40, 1:200, Dako), CD68 (Clone PG-M1, 1:100, Dako), and Ki-67 (Clone MIB-1, 1:400, Dako). S-100 was strongly expressed in 80% of the chondrocytes, whereas D2-40 was weakly expressed in 90%. CD68 and
Ki-67 were positive in 5% and v1%, respectively. The final diagnosis was Grade I chondrosarcoma. Because involvement of the superior margin was confirmed in the histopathologic examination, the patient received 54 Gy radiotherapy in 30 fractions. There was no evidence of recurrence or distant metastasis on CT and positron emission tomography (PET) scans at an 8-month follow-up visit (Fig. 5).
Discussion From 1954 to the present, 30 cases of TMJ chondrosarcoma have been reported in the English literature (Table 1).3–29 The age ranged from 7 to 75 years old, with the mean age of 46.5 years. The mean age of chondrosarcoma in TMJ was slightly higher than that in the head and neck.30,31 A female predominance, with a female-to-male ratio of 1.8:1, was found, which was not consistent with a male predominance in the head and neck or other body parts.30–32
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Figure 4 Histopathologic features of temporomandibular joint (TMJ) chondrosarcoma. Hematoxylin and eosin stains. (A) Lobular growth pattern of numerous lacunae within a chondroid matrix ( 3 100). (B) Chondrocytes showed marked pleomorphism, and a double-nucleated chondrocyte was found (arrow, 3 400). (C) Fibrous tissue septa separating coalesced lobules ( 3 200). (D) Tumor invasion into the medullary bone ( 3 200).
Figure 5 Panoramic radiograph at an 8-month postoperative follow-up.
Clinically, swelling was the most frequent symptom of TMJ chondrosarcoma, followed by pain, trismus, and hearing loss (Fig. 6). Headache, facial asymmetry, and paresthesia were each reported once. The major radiological features of TMJ chondrosarcoma were condylar resorption, erosion of adjacent bone, and calcification within the mass (Fig. 7). In three reported cases, more destructive bony change than erosion was detected in the external auditory canal, petrous temporal bone, and mandibular ramus.3,19,27 An enhancing peripheral rim was found in four cases and a periosteal reaction in two cases. Oliveira et al.22 mentioned the importance of MRI in the differential diagnosis of TMJ 4
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chondrosarcoma. In their case report, hypercellular content of the lesion and the presence of a chondroid or myxoid matrix were confirmed through MRI. In addition, MRI was recommended for preoperative planning because it provided detailed information on the anatomic limits and the most accurate view of the magnitude of the lesion.22,24 In the present case, however, condylar destruction, erosion of the cranial base, and mass architecture, including an enhancing peripheral rim and internal septa, were more distinctly found on CT than MRI. Given that these radiological findings could be clear bases suggesting chondrosarcoma rather than synovial cyst, CT as well as MRI is recommended for the accurate diagnosis and proper preoperative planning in TMJ chondrosarcoma. The time from the first perception of symptoms to the initial visit to the clinic ranged from 2 to 96 months in 26 cases. The mean time of 20.7 months in TMJ chondrosarcoma, approximately three times longer than that in head and neck chondrosarcoma,31 suggested the propensity for slow growth of the lesion, allowing for its malignancy, and should be considered by clinicians so as not to miss chondrosarcoma in the differential diagnosis. In 22 reported cases, the greatest diameter of TMJ VOL .
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Tullio (1974)9 Nortje´ (1976)10
Sato (1977)11
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18
Bernasconi (2004)20 Gallego (2009)21 Oliveira (2009)22 Garzino-Demo (2010)23
Mostafapour (2000)3
2015
VOL .
11
65
21
22
54
65
16
20
66
15
55
60
14
19
50
13
00
F
F
M
F
F
F
M
F
F
F
F
36
Merrill (1997)16b Sesenna (1997)17 Ichikawa (1998)18b Batra (1999)19
F
F
29
49
F
–
– –
60
–
– –
F M
F M
F
11 Wasenko (1990)14 15 12 Nitzan (1993)
9 Cadenat (1979)12 Morris (1987)13 10
8
48 75
2 3
17 40
46
1
Gingrass (1954)7 Lanier (1971)4a Richter (1974)8
First author
3
2
3
48
18
96
18
12
12
36
72
–
24
–
4
36 18
8 6
24 10
12
Swelling, pain
Swelling, pain, trismus Pain, trismus
Pain, trismus
Swelling
Swelling, pain, trismus Swelling, hearing loss Swelling
Swelling
Swelling, pain, trismus Trismus
Swelling, headache Swelling, pain
Pain Swelling, pain, trismus Swelling, pain, trismus Swelling, pain
Swelling, pain, trismus Swelling, pain Swelling, pain, hearing loss Swelling Swelling, pain
Duration of Case symptoms No. Age Sex (months) Chief complaints
Mass with calcification, condylar resorption
Mass with enhancing peripheral rim, condylar resorption
Mass adjacent to condyle
Mass with enhancing peripheral rim, adjacent bony erosion
Adjacent bony destruction
Mass with calcification, adjacent bony erosion and destruction Condylar resorption
Mass with calcification
Mass with calcification
TMJ space widening, condylar resorption, adjacent bony erosion Radiopacity with radiolucency
Mass with calcification
Mass with calcification, adjacent bony erosion
–
Radiopacity with radiolucency
Condylar resorption TMJ space widening, elongated condyle, adjacent bony erosion Condylar resorption TMJ space widening, elongated condyle, radiopacity with radiolucency, adjacent bony erosion – Radiopacity with radiolucency
Bone deposition
Radiological features
size
I
I
2.7|1.9 –
I
2.2|1.3|0.5
I
6.0|4.0|3.0
I
I
6.0|6.0
3.5
I
–
I
–
2.2|1.5|0.7, 1.3|1.0|0.5 4.0|1.8
–
4.0
I
I
4.0|2.5 2.5
I
–
–
– –
– I
– –
–
Surgery, RT
Surgery
Surgery
Surgery, RT after recurrence Surgery
Surgery
Surgery
Surgery
Surgery
Surgery
Surgery
Surgery
Surgery, RT
Surgery
Surgery
Surgery, RT Surgery
Surgery Surgery
Surgery Surgery
Surgery
Treatment Grade surgery
2.5
–
–
– –
– 6.0
2.0 6.0|5.0|3.5
–
Tumor (cm)
Table 1 Thirty cases of temporomandibular joint (TMJ) chondrosarcoma reported in the English literature and the present case
9 years
3.5 years
16 months
1 year
12 months, recurrence
–
7 months
3 years
5 years
18 months
7 years
–
6 months
4 months
–
– –
– 24 months
14 months 12 months
–
Follow-up
Oh et al. Case report of TMJ chondrosarcoma
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5
6
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RamosMurguialday (2012)25 Abu-Serriah (2013)26 Goutzanis (2013)27 Coleman (2013)6d Giorgione (2013)28 Kumar Reddy (2014)29 Present case (2014)
Gonza´lezPe´rez (2011)24 Xu (2011)5c
First author
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60
31
63
28
30
23
27
56
48
26
29
45
25
0
F
M
M
F
M
M
M
F
2
2
36
12
12
–
–
–
Swelling, pain, trismus, paresthesia
Swelling, pain, trismus Swelling, pain
Swelling
Swelling, pain
Pain
Trismus, facial asymmetry Asymptomatic
Lobulated mass with enhancing peripheral rim and internal septa, condylar resorption, adjacent bony erosion, periosteal reaction
Mass with calcification, condylar resorption, periosteal reaction Mass with calcification, condylar resorption
Lobulated chondroid mass, condylar resorption
Condylar resorption, adjacent bony destruction
Mass with calcification, adjacent bony erosion
Mass with calcification, condylar resorption, adjacent bony erosion Multilocular radiolucency with mandibular osteolysis
34
24
Swelling, pain
Mass with enhancing peripheral rim, condylar resorption
12
57
23
M
Radiological features
Duration of Case symptoms No. Age Sex (months) Chief complaints
Table 1 Continued
I
I
5.0|3.0 5.3
I
–
5.5|5.0|4.0 4.6|4.0|3.9
I
6.0
–
II
5.5|2.0 1.0
I
8.0|6.0|5.0
Surgery, RT
Surgery
Surgery, RT
Surgery, RT
Surgery
Surgery
Surgery
Surgery
Surgery
Treatment Grade surgery I
size
–
Tumor (cm)
5 months
1 year
–
–
2 years
6 months
3 years
–
2 years
Follow-up
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Oh et al.
Case report of TMJ chondrosarcoma
Figure 6 Chief complaints of 28 cases of TMJ chondrosarcoma in the English language literature.
Figure 7 Radiological features of 29 cases of TMJ chondrosarcoma in the English language literature.
chondrosarcoma ranged from 1.0 to 8.0 cm, with the mean size of 4.3 cm. The tumor size of chondrosarcoma in 2TMJ was similar to that in the skull base but smaller than that in the head and neck.31,33 Temporomandibular joint chondrosarcoma has typical histopathologic features of chondrosarcoma in that lacunae, including pleomorphic chondrocytes, show a lobular growth pattern within the chondroid matrix, and double-nucleated nuclei are occasionally detected. According to Evans et al.,32 chondrosarcoma has been classified into three grades on the basis of histologic criteria, including nuclear size, mitosis, cellularity, and matrix character. In this review on histologic grading of TMJ chondrosarcoma, by classifying ‘well-differentiated’ or ‘low grade’ into ‘Grade I’, 18 out of 19 cases were diagnosed as Grade I, with only one as Grade II. This demonstrated that the percentage of Grade I is much higher in TMJ chondrosarcoma than in other body parts.32,34 Malignant transformation from a benign lesion, including synovial chondromatosis, multiple chondroma, or osteochondroma, to chondrosarcoma has been reported as secondary chondrosarcoma.35 In previous studies, the malignant transformation rate in synovial chondromatosis was 5–10% and
that of solitary osteochondroma was 0.4–2%.35–37 In reports of TMJ chondrosarcoma, three cases of secondary chondrosarcoma arose from synovial chondromatosis, osteochondroma, and enchondroma, respectively.4–6 In the present case, the initial diagnosis was a synovial cyst, based on the MRI scan. Although the diagnosis was not definitive because a biopsy was not performed at that time, synovial cyst was the most probable diagnosis, based on the images showing the cystic lesion with synovial enhancement and the fluid from the aspiration. As treatment for a synovial cyst, an arthroscopic procedure instead of surgical excision was suggested for its conservativeness and management of inflammation.38 However, if the cyst does not disappear spontaneously after the arthroscopic procedure, surgical excision should be carried out to prevent aggravation of the lesion. Although malignant transformation of synovial cysts has not been reported to date, clinicians are advised to notice the possibility of malignant transformation, taking the present case into account. Out of 31 cases of TMJ chondrosarcoma, fine needle aspiration biopsy (FNAB) was performed in eight cases. Among these cases, the diagnosis of chondrosarcoma could be inferred from FNAB in
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only two cases; in one of these cases, the second FNAB was necessary for diagnosis due to inadequacy of the first biopsy.19,23 In four cases, FNAB was not indicative of chondrosarcoma20,24,27 or insufficient for definitive diagnosis.13 Two of the cases reported that the result of FNAB was pleomorphic adenoma.17,28 In the present case, a small punch biopsy was performed, and the result implied cartilage proliferation but was not sufficient for definitive diagnosis as chondrosarcoma. A small amount of tissue from FNAB or a punch biopsy in chondrosarcoma shows pleomorphic chondrocytes and double-nucleated nuclei, but these cellular features can also be seen in other chondroid lesions like synovial chondromatosis.39 To take advantage of FNAB in TMJ lesions suspicious for chondrosarcoma, immunohistochemical stains may play a major role in reaching a reliable diagnosis. To date, only six cases of TMJ chondrosarcoma had immunostaining.17,18,21,22,24,28 In the results, S-100 was positively expressed in 5–35% of chondrocytes17,21,24 and Ki-67 was in 5–50%.21,24,28 Compared to these results, the present case showed high expression of S-100 and low expression of Ki-67. Additionally, D2-40 and CD68 immunostains were carried out in the present case. D2-40 is a known marker of cartilaginous tumors and has been studied for its expression in chondrosarcoma.40–42 CD68 expression in chondroid lesions has been reported in two papers. One paper showed negative CD68 expression in clear cell chondrosarcoma as well as in conventional chondrosarcoma.43 The other paper reported positive expression in a few chondrocytes of primary synovial chondromatosis.44 In-depth studies of immunostains in chondrosarcoma compared to other chondroid lesions are necessary for accuracy in differential diagnosis, especially in cases of a small biopsy such as FNAB. Due to the absence of lymph node metastasis on preoperative imaging, all patients with TMJ chondrosarcoma were treated surgically without neck dissection. In 7 out of 31 TMJ chondrosarcoma cases, radiotherapy was performed after surgery and the mean dose was 63 Gy, averaged from doses reported in three cases, including the present case.13,23 Although chondrosarcoma had traditionally been regarded as a radioresistant tumor, Harwood et al.45 concluded that chondrosarcoma of the bone is a radioresponsive tumor. Burkey et al.46 insisted that postoperative radiotherapy can be useful in some extensive, high grade, or incompletely resected chondrosarcomas. Only one case of TMJ chondrosarcoma reported a recurrence 1 year after surgery.3 The patient in the 8
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case declined radiotherapy despite positive margins in the histopathologic analysis. Considering its efficacy as mentioned above, postoperative radiotherapy should be applied to cases of TMJ chondrosarcoma with incomplete surgical resection margin.
Disclaimer Statements Contributors K-YO, as a first author, contributed to selecting the case and writing the article in whole. H-JY, J-IL, and S-PH contributed in reviewing the literature and revising the article in part. S-DH, as a corresponding author, contributed in directing all the process and revising finally.
Funding Conflicts of interest There are no conflicts-of-interest.
Ethics approval Not required. Because this is a report of a single case not including private information such as patient’s identification and face photos.
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