Mesenchymal Chondrosarcoma of the Jaws: a Series of Two Rare Cases

10.5005/jp-journals-10011-1265 SatyaCASE Ranjan REPORT Misra et al Mesenchymal Chondrosarcoma of the Jaws: A Series of two Rare Cases

Satya Ranjan Misra, G Maragathavalli, Vineet Daniel Alex, Sobhan Mishra

ABSTRACT Panoramic radiography revealed decreased radiodensity Chondrosarcoma (CS) is a malignant neoplasm that results in in the right ramus, with erosion of the condylar outline. abnormal bone and cartilage growth. Although CS is rare, it is There was a typical ‘sunburst’ appearance in the condyle the second most common primary bone malignancy. extending up to the angle of the mandible on the right side Mesenchymal chondrosarcoma (MC) is a rare histological variant of CS that accounts for only about 1% of all CS and has (Fig. 2). high predilection for the head and neck region. It is usually seen CT scan revealed osteosclerosis and erosion of the in younger age group compared to conventional CS and the condyle extending upto the ramus, a nonenhancing mass maxillary alveolus is the most common site. The tumor is unique because of its aggressive growth with a high tendency for late with mottled calcifications was seen in the axial sections recurrence and delayed metastasis. We present two cases of measuring 6.4 × 6.5 cm. The classic ‘sunburst’ appearance MC, one case involving the mandibular condyle and the second, is also seen. The coronal images also show extension of the a metastatic CS with the primary in the clavicle. involvement of the marrow space (Figs 3 and 4). Keywords: Chondrosarcoma, Mesenchymal chondrosarcoma, An incisional biopsy was then done; histological Metastasis. examination revealed highly cellular connective tissue How to cite this article: Misra SR, Maragathavalli G, Alex VD, stroma with formation of large amounts of chondroid matrix Mishra S. Mesenchymal Chondrosarcoma of the Jaws: A Series of two Rare Cases. J Indian Aca Oral Med Radiol (Fig. 5). The nuclei of the chondrocytes were large 2012;24(1):78-82. and pleomorphic with an open chromatin pattern (Fig. 6). Source of support: Nil Conflict of interest: None declared

CASE REPORTS Case 1 A 22-year-old male patient reported to the dental hospital with the complaint of a swelling on the right side of the face since the past 2 months. History revealed that the swelling has been progressively growing in size over the period of 2 months. An ulcer developed in the right buccal mucosa about 1 month back which did not heal with medications. The patient had a restricted mouth opening and numbness in the right side of the lower lip since last 15 days. On examination, a diffuse swelling was seen causing facial asymmetry on Fig. 1: Ulceroproliferative growth was seen in the right retromolar region the right side of the face, ovoid in shape, measuring 7 × 6 cm in size. The swelling was firm in consistency, fixed to the underlying bone and nontender on palpation. No secondary changes of ulcer or sinus were seen and there was no discharge. Intraorally, an ulceroproliferative growth was seen in the right retromolar region extending anteriorly upto 45, measuring about 3 × 3 cm, the ulcer was covered with slough, with irregular margins. It was indurated and tender on palpation. There was no discharge from the ulcer, surrounding area firm in consistency with bicortical expansion of the mandible in the 45 to 47 region. A provisional diagnosis of a malignant bony neoplasm was Fig. 2: Sunburst appearance in the condyle extending up to the given (Fig. 1). angle of the mandible 78 JAYPEE JIAOMR

Mesenchymal Chondrosarcoma of the Jaws: A Series of two Rare Cases

There was evidence of cytoplasmic vacuolization and the Immunohistochemistry was done and the chondrocytes were presence of typical binucleate forms and there numerous positive for S100. Histopathology was suggestive of MS. mitotic figures with few foci of calcification. The patient underwent surgery and chemotherapy.

Case 2 A 31-year-old male patient reported to the dental hospital, with the chief complaint of swelling and pain in left posterior maxilla for the past 2 months. History revealed that the swelling was started as a small one and gradually grew to the present size. He also had pain while swallowing. There was the history of a similar painful swelling in the left clavicle 6 months back for which he had undergone surgery (Fig. 8). On examination, an ulceroproliferative growth was seen in the left maxillary alveolus measuring approximately 6 × 4 cm in size, extending from the left maxillary premolar region into the tuberosity and soft palate (Fig. 7). There Fig. 3: Axial CT scan shows osteosclerosis and erosion of the were surface lobulations with ulceration posteriorly, covered condyle extending up to the ramus, a nonenhancing mass with by a greyish white slough. It was firm in consistency and mottled calcifications with the sunburst appearance tender on palpation. The lesion was provisionally diagnosed as a malignant neoplasm. Panoramic radiography revealed decreased radiodensity in the left maxillary alveolus with destruction of the floor of the maxillary sinus and soft tissue mass in the sinus. There was the evidence of patchy radiopacities in the otherwise osteopenic lesion. CT scan revealed a 5.3 × 6.2 × 6 cm hypodense region with specks of hyperdense areas, eroding the posterior maxillary wall. Tumor infiltration into the retromaxillary region, pterygoid musculature and lateral wall of the nose was evident. A space occupying lesion seen eroding the lateral and medial walls of the maxillary antrum on the right side and almost filling it. The CT images were

Fig. 4: Coronal CT scan showing the involvement of the ramus suggestive of an aggressive malignant lesion (Figs 9 and condyle, marrow spaces are also infiltrated and 10).

Fig. 5: Histopathology revealing highly cellular connective tissue Fig. 6: Large and pleomorphic nuclei of the chondrocytes with stroma with formation of large amounts of chondroid matrix an open chromatin pattern Journal of Indian Academy of Oral Medicine and Radiology, January-March 2012;24(1):78-82 79 Satya Ranjan Misra et al

An incisional biopsy was done and histopathology MC is a rare histologic type of CS that was first described revealed characteristic bimorphic pattern composed of by Lichenstein and Bernstein in 1959 as a biphasic tumor sheets of undifferentiated round and spindle cells along with with areas comprising of spindle cell mesenchyme areas of chondroid matrix of variable cellularity (Fig. 11). Cells of chondroid differentiation exhibited both nuclear and cytoplasmic pleomorphism, hyperchromasia along with numerous abnormal mitotic figures. Areas of ossification were also evident. Immunohistochemistry was done and the chondrocytes were positive for S100 (Fig. 12). Histopatho- logical features were suggestive of MC. Correlating with the surgical history and clinical findings, a final diagnosis of MC of maxilla metastasized from clavicle was made. The patient underwent surgery but had recurrence in the clavicle and later passed away before he could undergo any further treatment.

DISCUSSION

Phemister in 19301 defined chondrosarcomas as sarcomas Fig. 8: Surgical scar in the left clavicular region of bone containing abundant cartilage. Lichtenstein and Jaffe2 established the criteria for classification of chondrosarcomas. They defined chondrosarcomas as arising from full-fledged cartilage and never containing osteoid or bone stroma. According to the International Classification of Diseases for Oncology,3 CS has been classified into six subtypes, on the basis of the tumor location, the histological characteristics of the malignant cartilage cells, and the makeup of the surrounding matrix material associated with the tumor are as follows: 1. Chondrosarcoma NOS (Not otherwise specified) 2. Juxtacortical chondrosarcoma 3. Myxoid chondrosarcoma 4. Mesenchymal chondrosarcoma

5. Clear cell chondrosarcoma Fig. 9: Axial CT scan showing a hypodense mass with specks of 6. Dedifferentiated chondrosarcoma hyperdense areas, eroding the posterior maxillary wall

Fig. 7: Ulceroproliferative growth in the left maxillary Fig. 10: Coronal CT scan showing a space occupying lesion seen alveolus eroding the lateral and medial walls of the maxillary antrum on the right side and almost filling it 80 JAYPEE JIAOMR

Mesenchymal Chondrosarcoma of the Jaws: A Series of two Rare Cases

Chondrosarcomas do not show any gender predilection. The tumor can occur between 10 and 80 years of age.8 However, it is more common during the second and third decades of life, therefore it is a malignancy of the young individuals as seen in our cases. There are no specific clinical signs or symptoms but the first clinical symptom is a painless swelling or mass, rapidly growing to a large size and leading to facial deformity, nasal obstruction, expansion of the bone and its subsequent perforation as it enlarges, surface ulceration may also be seen.9 Later symptoms include pain and mobility of the teeth in the region. Rarely facial paralysis can also occur.10 Radiographically, chondrosarcoma is seen as an Fig. 11: Histologic section showing characteristic bimorphic pattern irregularly shaped, ill-defined radiolucency with randomly composed of sheets of undifferentiated round and spindle cells along scattered, opaque mottled areas. The lesion may appear with areas of chondroid matrix of variable cellularity with areas of ossification lobulated and may elevate the adjacent periosteum to produce radiating laminae in a sunray pattern as seen in the first case. Perforation of the cortex of the involved bone by a cartilaginous lesion is considered a sign of probable malignancy. Since these are not pathognomonic signs of chondrosarcoma the radiographic differential diagnosis also includes fibrosarcoma and osteosarcoma. Evaluation of the maxillary region on conventional radiographs is somewhat limited due to anatomical superimposition and two dimensional displays.11 CT performed with contrast enhancement is the imaging modality of choice because it best delineates the bony involvement while defining local invasion into the adjacent soft tissues as seen in both our reported cases.12 Depending on the cellularity, nuclear staining (hyperchromasia) of the tumor cells and size of the nuclei, Fig. 12: Immunohistochemistry showing the chondrocytes were 13 positive for S100 chondrosarcomas have been graded into three types: • Grade I (or low grade): These tumors are characterized interspersed with areas of chondroid differentiation by the presence of benign cartilage, have a relatively accounting for only about 1% of all chondrosarcomas.4 It uniform and lobular histologic appearance. Presence of develops from a pluripotential mesenchymal stem cell which atypical cells including binucleate forms may also be can differentiate into angioblastic, fibroblastic and recorded. cartilaginous structures.5 • Grade II (or intermediate grade): These tumors are Approximately one-third of mesenchymal chondro- characterized by a higher cellularity with a greater degree sarcomas are found outside bone within a variety of soft of nuclear atypia, hyperchromasia with often having tissues, while two-thirds of these tumors are intraosseous. myxoid stroma and enlarged chondrocyte nuclei. Most chondrosarcomas of the head and neck region occur • Grade III (or high grade): These tumors are characterized in the maxilla; others are seen to occur less commonly in by a higher cellularity, marked cellular and nuclear the body of the mandible, the ramus, the nasal septum and pleomorphism, nuclear hyperchromasia and increased the paranasal sinuses.6 In the maxilla, the most common mitosis with occasional presence of giant cells. location is anterior alveolus where preexisting nasal Histologically, MC has two characteristic components. cartilage is present. Both our reported cases are extremely The first is a dense population of anaplastic small cells either rare as one is in the mandibular ramus with extensive in solid sheets or in a hemangiopericytoma-like pattern and condylar involvement7 and the other in the posterior maxilla the second is marked by a characteristic chondroid matrix. metastasizing from the clavicle. Both our cases were characteristic of MC as dense Journal of Indian Academy of Oral Medicine and Radiology, January-March 2012;24(1):78-82 81 Satya Ranjan Misra et al population of anaplastic cells in a chondroid matrix were 8. Shafer’s textbook of oral pathology, Rajendran, Sivapatha- seen and since there was marked cellular and nuclear sundaram. Benign and malignant tumours of the oral cavity (6th ed). Elseivers 2009;162-65. pleomorphism, nuclear hyperchromasia; both cases were 9. Niven Tien, et al. Mesenchymal chondrosarcoma of the maxilla: considered high grade. Immunohistochemistry also can be Case report and literature review. J Oral Maxillofac Surg done for difficult cases, chondroid areas are positive for 2007;65:1260-66. S100 protein and neurone-specific enolase is focally positive 10. Kikuo takahashi, et al. Mesenchymal chondrosarcoma of the 14 jaw-report of a case and review of 41 cases in the literature. for primitive mesenchymal cells. Both our cases were S100 Head and Neck 1993;15:459-64. positive for chondrocytes. 11. Hayt MW, Becker L, Katz DS. Chondrosarcoma of the maxilla: Wide surgical excision is the mainstay of treatment for Panoramic radiographic and computed tomographic with MC in the jaw bones.15 These tumors are radioresistant and multiplanar reconstruction findings. Dentomaxillofacial Radiology 1998;27:113-16. chemotherapy can be used as an adjuvant therapy after wide 12. Jaetli Vipul, Gupta Sunita. Mesenchymal chondrosarcoma of 16 surgical excision is made. Our cases were treated with maxilla: A rare case report. Med Oral Patol Oral Cir Bucal wide surgical excision followed by chemotherapy, but the 2011 July 1;16 (4):e493-96. second case, the primary was in the clavicle which had 13. Puri Ajay, et al. Chondrosarcoma of the bone, does the size of the tumour, presence of pathologic fracture, or prior intervention recurrence and the patient passed away. Generally the have an impact on local control and survival? J Cancer Res prognosis of MC is considered to be poor and the grade, Ther 2009 Jan;5(1). the size of the tumor, the adequacy of tumor resection 14. Kumaraswamy, et al. Mesenchymal chondrosarcoma affecting margins may be prognostic indictors. Local recurrence the mandible. Journal of Oral and Maxillofacial Pathology 2008 Jan-Jun;12(1). occurs and may indicate subsequent metastasis; hence 15. Saini Rajan, Abd Razak Noor Hayati, Shiafulizan Ab Rahman, adequate treatment and long-term follow-up, including Abdul Rani Samsudin. Chondrosarcoma of the mandible: A case periodic systemic evaluation, are required for patients with report. JCDA 2007 March;73(2). MC of the maxillofacial region. 16. Krishnamurthy, et al. A fatal case of mesenchymal chondro- sarcoma of the mandible. J Cancer Res Ther 2011 Jan;7(2): 192-94. REFERENCES

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