Fibrosarcoma of the Temporomandibular Joint Area: Benefits of Magnetic Resonance Imaging and Computed Tomography

Vol. - No. - Month 2014

Fibrosarcoma of the temporomandibular joint area: bene ﬁts of magnetic resonance imaging and computed tomography Shoko Gamoh, DDS, PhD, a Yukako Nakashima, DDS, a Hironori Akiyama, DDS, PhD, a Kaname Tsuji, DDS, PhD, b Koji Yamada, DDS, PhD, b Motoyuki Suzuki, MD, PhD, c Shosuke Morita, DDS, PhD,b and Kimishige Shimizutani, DDS, PhD a Osaka Dental University and Osaka Medical Center for Cancer and Cardiovascular Diseases, Osaka, Japan (Oral Surg Oral Med Oral Pathol Oral Radiol 2014;-:1-5)

Fibrosarcoma is a malignant neoplasm composed of (Figure 4, A, B). CT found signi ficant bony erosion of the fibroblasts that produce collagen and elastin. It may inner cortex of the left ramus of the mandible ( Figure 5). The arise either in the soft tissue or in bone, with the latter patient was referred to another hospital for further manage- comprising 20% of all cases. 1,2 The osseous origin of ment because the tumor grew toward the pharyngeal arches. fibrosarcomas has been debated since 1940, when An incisional biopsy was subsequently performed, and the “fi ” initial diagnosis was a sarcoma based on the histopathologic Ewing named the entity broblastic sarcoma, but the fi 3 examination of the biopsy. A de nitive histologic diagnosis concept has been generally accepted. Fibrosarcomas in was reached by exclusion. The tumor was diagnosed as a the masticator space can be diagnostically challenging. grade 3 (poorly differentiated) fibrosarcoma using the A total of 76 cases of fibrosarcoma in the jaw exist in FNCLCC (Fédération Nationale des Centres de Lutte Contre 4 the English-language literature from before 2007. le Cancer) grading system. Histopathologic examination Here, we report a case of primary fibrosarcoma in the found that the tumor contained spindle cells arranged in mandible of a 33-year-old woman with symptoms and compact fascicles that were intersected by various amounts of radiologic signs mimicking temporomandibular disor- delicate thin to dense keloid-like collagen. Cell bundles were der. We also present a literature review of fibrosar- arranged at acute angles to each other, whereas the presence comas in the jaw. of fascicles was subtler in other areas. A prominent storiform pattern was not seen (Figure 6). The fibrosarcoma was resected followed by postoperative radiotherapy and chemo- CASE REPORT therapy. The tumor was removed, along with the left ramus of A 33-year-old woman presented with a 1-year history of the mandible, a portion of the left maxilla, a deep part of the trismus associated with a clicking noise in the left temporo- parotid gland, and lymph nodes located superior to the mandibular joint (TMJ). Her chin shifted to the right side omohyoid muscle. The cheek mucosa was reconstructed using when she opened her mouth (Figure 1). Panoramic radio- a free anterolateral thigh flap ( Figure 7). The patient recovered graphs (Figure 2, A, B) and magnetic resonance imaging and got married afterward; however, lung metastasis with (MRI) scans (Figure 3, A-H) of the TMJ regions showed signs of temporomandibular disorder (TMD), so conventional treatment for TMD was prescribed, including occlusal splints, mouth-opening exercises, and a muscle relaxant for 6 months. When the MRI scout images were analyzed retrospectively, the findings of 2 of 5 scout images were suggestive of tumor (see Figure 3, A, E). The patient was referred again 6 months after the initial visit for investigation of insomnia caused by spontaneous pain in the left TMJ area. She also had paralysis of the lower lip and left chin. She had herpes labialis and gastroenteritis caused by stress. Given the suspicion of malignancy, both MRI and computed tomography (CT) were performed. MRI showed a round, inhomogeneous mass in the pterygomandibular portion of the masticator space, which caused minimal medial narrowing of the left parapharyngeal space Fig. 1. Facial photograph. The patient ’s chin shifted to the right side when she opened her mouth. aDepartment of Oral Radiology, Osaka Dental University. bFirst Department of Oral and Maxillofacial Surgery, Osaka Dental University. cDepartment of Otolaryngology, Osaka Medical Center for Cancer Statement of Clinical Relevance and Cardiovascular Diseases. Received for publication Aug 23, 2013; returned for revision Nov 29, 2013; accepted for publication Feb 4, 2014. There are significant pitfalls of relying on a pano- Ó 2014 Elsevier Inc. All rights reserved. ramic radiograph when patients have symptoms of 2212-4403/$ - see front matter temporomandibular disorders. http://dx.doi.org/10.1016/j.oooo.2014.02.013

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Fig. 2. Panoramic radiographs. Images of panoramic ( A) and panoramic temporomandibular joint projection ( B). Findings showed reduced movement of the right temporomandibular joint.

Fig. 3. Magnetic resonance imaging (MRI) at the initial visit. A routine MRI protocol for temporomandibular disorders was chosen. Key images from each series are shown. A, E, Scout images. B, T2-weighted sagittal image of the right temporomandibular joint (TMJ) on occlusion. C, T1-weighted sagittal image of the right TMJ with the mouth opened maximally. D, T1-weighted coronal image of the right TMJ on occlusion. F, T2-weighted sagittal image of the left TMJ on occlusion. G, T1-weighted sagittal image of the left TMJ with the mouth opened maximally. H, T1-weighted coronal image of the left TMJ on occlusion. chest wall invasion was detected 3 years after the surgical accounts for about 5% to 6% of all adult soft tissue procedure. After further surgery and chemotherapy for the sarcomas.5-8 According to Leitner et al., 9 fibrosarcomas lung metastasis, the patient remains well with no signs of constitute approximately 10% of sarcomas in the head recurrence. and neck region, with the mandible being the most common site of occurrence. Fibrosarcomas may arise in DISCUSSION any part of the jaw and are classi fied as either peripheral Fibrosarcoma is a rare malignant tumor of fibroblastic or central type. 10,11 Fibrosarcoma of the bone may be origin that particularly affects the long bones and associated with conditions such as fibrous dysplasia12 OOOO CLINICOPATHOLOGIC CONFERENCE Volume -, Number - Gamoh et al. 3

Fig. 4. Magnetic resonance imaging scans. A, Axial view. B, Coronal view. Arrows point to a round inhomogeneous mass sized 40 mm in diameter in the pterygomandibular portion of the masticator space. The lesion caused minimal medial narrowing of the left parapharyngeal space.

Fig. 6. Histopathologic ﬁndings. Spindle cells were arranged in compact fascicles intersected by various amounts of delicate thin to dense keloid-like collagen.

Fig. 5. Computed tomography image. The arrow points to the Histologically, fibrosarcomas are composed of a region where bony erosion was observed in the medial cortex uniform population of spindle-shaped cells arranged in of the left mandible. a fascicular or herringbone growth pattern with a vari- able amount of collagen production. Given that these and Paget disease and may arise after radiotherapy. 4 “fibrosarcomatous ” features can be seen in a wide va- Onset is usually before the age of 50, with a peak riety of tumors, the majority of tumors previously incidence between 20 and 40 years, but it may occur at considered to reside in this category are probably better any age.13 They usually present as an asymptomatic, placed in other categories. Thus, fibrosarcoma is a firm mass in the soft tissue of the face, neck, scalp, or diagnosis of exclusion that cannot be made using a paranasal sinuses 14 and may grow to a considerable size limited amount of tissue. 20 In the present case, we could before causing symptoms. Symptoms include pain, not reach a de finite histologic diagnosis without an swelling, loosening of the teeth, paresthesia, and oc- excisional biopsy. Other malignant tumors (such as casionally ulceration of the overlying mucosa. 15-17 metastatic carcinoma, multiple myeloma,18 monophasic Involvement of the TMJ or paramandibular muscula- fibrous synovial sarcoma, malignant fibrous histiocy- ture is often accompanied by trismus. 18 toma, malignant nerve sheath tumor, and liposarcoma) Most fibrosarcomas appear radiographically as lytic must be considered in the differential diagnosis, as well lesions with a destructive pattern. The cortex is thinned as benign tumors such as benign fibrous histiocytoma, or disrupted, and soft tissue invasion is detected in up to fibroma, and fibromatosis.10 If a suspected fibrosarcoma 86% of cases. 4,6,7 However, fibrosarcoma of the jaw enlarges the affected jaw, the practitioner must rule out cannot be distinguished from other conditions that chondrosarcoma and osteosarcoma, both of which have cause destructive lesions in the bone. 19 a de fined internal structure.18 CLINICOPATHOLOGIC CONFERENCE OOOO 4 Gamoh et al. Month 2014

Fig. 7. Surgical ﬁndings. A, Design of the surgical procedure. Gray area is surgically removed. B, Site of reconstruction using microsurgery. The arrow indicates free anterolateral ﬂap taken from the patient ’s left thigh.

Table I. Case reports of ﬁbrosarcomas of the jaw Authors Year Age (y) Sex Site Maximal opening Treatment Recurrence Follow-up Status Herrera et al. 14 2007 72 F Mandible 40 mm Surgery None 3 mo Alive Angiero et al. 30 2007 53 M Mandible NA Surgery Local þ lung 3 y Dead 71 M Maxilla NA Surgery None 5 y Alive Orhan et al. 29 2007 14 F Mandible 17 mm Surgery þ RT þ CT None 3 mo Alive Pereira et al. 4 2005 41 F Mandible NA Surgery þ RT None 3 y Alive NA , not available; RT , radiotherapy; CT , chemotherapy.

Wide surgical excision is the mainstay treatment for a of a clicking noise is a frequent manifestation of TMD. fibrosarcoma, with overall survival rates of 45% to In TMD, joint noises are accompanied by a restriction 82%.21-24 Despite minimal bene fit, radiochemotherapy of mandibular mobility and joint pain. The character- is recommended in cases of incomplete resection with istic feature in our case was the acute, spontaneous pain positive margins or high-grade tumors with locore- in the left TMJ, but reduced joint movement was found gional spread.22 Complete resection with clear surgical only in the right TMJ on radiography; these features did margins is associated with a favorable prognosis. 24-26 not fit those of TMD. Speci fically, panoramic visuali- Soames and Southam 12 stated that fibrosarcoma of the zation of the TMJ found that restricted movement oral soft tissues is rare but appears to have a good occurred on only the right side. Careful observation prognosis, with reported 5-year survival rates of about found that the right condyle rotated and rolled, whereas 70%. Distant metastasis is rare, with the lungs being the the left condyle merely rolled without rotating. These most common site of metastasis, 27,28 as was seen in the findings were unusual for TMD, assuming that “TMD” present case. represents symptoms caused by abnormalities of the Fibrosarcoma of the jaw is rare; few cases have been masticatory muscles or joint capsule, disk displacement, reported. Orhan et al. 29 reported a case of primary and condyle deformities. TMD is de fined as “a collec- fibrosarcoma affecting the mandible and its surrounding tion of medical and dental conditions affecting the structures in a 14-year-old girl presenting with signs temporomandibular joint (TMJ) and/or the muscles of and symptoms similar to those of TMD. Herrera et al. 14 mastication, as well as contiguous tissue compo- described a 72-year-old woman with a left parotid, low- nents.”31 We usually diagnose TMD if the patient ex- grade, mucoepidermoid carcinoma and a myxoid periences trismus, clicking or crepitation noise in the fibrosarcoma of the condylar head of the left ramus of TMJ, or pain on mouth opening, mastication, or oc- the mandible who presented with symptoms similar to clusion. Considering the history of trismus and clicking those found in TMD. Angiero et al. 30 described 2 men, noise followed by jaw deviation, a provisional diag- aged 53 and 71 years, with primary fibrosarcoma of the nosis of TMD because of nonreducible disk displace- jaw with intraosseous growth. In the present case, the ment was made in the present case, and initial periosteal origin of the lesion made its detection on radiologic examination was performed based on this radiographs difficult, and it exerted pressure on the assumption. The deviation of the jaw might suggest the medial surface of the mandible. existence of a space-occupying lesion. The jaw can also Case reports of fibrosarcomas of the jaw are sum- be shifted from the clicking side toward the contralat- marized in Table I. Among these reports, only the cases eral side in cases of TMD. Notably, jaw deviation was described by Herrera et al., 14 Orhan et al., 29 had present when the patient opened her mouth maximally, symptoms that mimicked those of TMD. The presence which is not possible in TMD. Enlargement of the OOOO CLINICOPATHOLOGIC CONFERENCE Volume -, Number - Gamoh et al. 5 tumor during the 6-month period, as shown by the 13. Som PM, Curtin HD, eds. Head and Neck Imaging. 4th ed. St difference in tumor size between the initial and subse- Louis, MO: Mosby; 2003. quent MRI (see Figures 3 and 4), most likely caused the 14. Herrera AF, Mercuri LG, Petruzzelli G, Rajan P. Simultaneous occurrence of 2 different low-grade malignancies mimicking spontaneous pain in the left TMJ area. As shown in temporomandibular joint dysfunction. J Oral Maxillofac Surg . Figure 5, the close proximity of the tumor to the 2007;65:1353-1358. mandibular canal might have caused paralysis of the 15. Handlers JP, Abrams AM, Melrose RJ, Milder J. Fibrosarcoma of lower lip and left chin. the mandible presenting as a periodontal problem. J Oral Pathol . Signs and symptoms of TMD could be caused by a 1985;14:351-356. 16. Sadoff RS, Rubin MM. Fibrosarcoma of the mandible: a case variety of intra- and extra-articular conditions, thus report. J Am Dent Assoc . 1990;121:247-248 . making the diagnosis of pain and dysfunction of the 17. Soares AB, Lins LH, Macedo AP, Pereira-Neto JS, Vargas PA. masticatory system complex. Our case illustrated that Fibrosarcoma originating in the mandible. 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