Mesenchymal Chondrosarcoma of the Mandible

Mesenchymal chondrosarcoma (MCS) was first described by Lichtenstein and Bernstein in 1959 as a special variant of chondrosarcoma [1]. It is a rare malignant tumor of bone and soft tissue. One of the most affected regions is the facial skeleton, especially the jaw. Males are more commonly affected [2]. Pain may be a late stage feature, and regional lymphadenopathy is very rare [3]. The lesion was originally grouped with osteosarcoma. These neoplasms are characterized by sheets or clusters of highly undifferentiated, small, ovoid cells that alternate with small zones of neoplastic cartilage. The prognosis for patients with MCS is unpredictable. This type of neoplasm shows aggressive local behavior as well as a high metastatic potential. Due to these features and the high risk of recurrence, the prognosis is poor.

A 20-year-old female presented to the surgical out-patients department with a large swelling of the right jaw. It was painless and gradually increased in size over a six and a half months period. The swelling measured about 15×10 cm, being fixed to the mandible and the soft tissues especially the cheek with intra-oral extension (Figure 1). The overlying skin was stretched and shiny. There were no engorged veins. The facial nerve was not involved with the tumor. The fixity to the masseter muscle could not be ascertained because of the large size of the tumor. There was no cervical lymphadenopathy. On abdominal examination, the liver was not palpable. There was no clinical evidence of distant metastasis. Fine needle aspiration cytology was characterized by the presence of isolated and loosely cohesive aggregates of oval or polygonal chondrosites with vacuolated granular cytoplasm and a background of chondroid matrix (Figure 2). A preoperative diagnosis of chondrosacoma of the mandible was made. The patient was given doxorubicin-based combination neo-adjuvant chemotherapy. The patient was then operated on. A left hemimandibulectomy was performed with wide surgical margins under general anesthesia. The postoperative course was uneventful. The contour of the face was maintained with the help of autologus rib graft (Figure 3). Postoperative radiation therapy was also given to the patient. The resected specimen showed the tumor along with the mandible (Figure 4). The histopathological examination was characterized by sheets of highly undifferentiated, small, ovoid cells having nuclear atypia, hyperchromasia with myxoid stroma. The diagnosis of mesenchymal chondrosarcoma was established. Three months after surgery, the patient was without local recurrence or metastasis.

Patients with malignant bone tumors often report at a very late stage of the disease [4]. Chondrosarcoma has a very aggressive course locally and tends to recur or metastasize in the late stages and therefore the long-term prognosis becomes extremely grave [4]. It may occur at any age; however, most frequently it is found between the third and sixth decades of life. Males are more commonly affected than females [2]. A study of all cases of maxillofacial chondrosarcomas revealed the maxilla to be more commonly involved than the mandible [5]. Our case, although being present in a young patient, was seen in the mandible and the diseased was a female highlighting the rarity of the lesion. Painless swelling of the jaw is the presenting symptom in most of these patients [6, 7]. In developing countries, patients with these tumors mostly report late for treatment and this period ranges between two weeks to four years with an average of 18 months [4]. Fine needle aspiration cytology (FNAC) of these lesions due to their rarity and limited experience with the spectrum of cytological and histopathological features poses a challenge to many cytopathologists. FNAC diagnosed 87.2% of jaw chondrosarcomas in a series by Nanda et al [8]. Resection with wide margins of clearance of the tumor is the agreed treatment of choice and gives the best outcome for the patients [9, 10]. Although preoperative chemotherapy and postoperative radiation therapy as adjunctive modalities were given to our patient, both these modalities have undefined roles and offer no survival benefits [7, 9].These tumors tend to recur locally after resection and metastasize even years after surgery. The most common site of distant metastasis is the lung. Crawford et al [9] noted that these tumors tend to recur or metastasize and found the five-year survival rate to be misleading. Overall, there is a ten-year survival rate of 28% [9]. Therefore, even after complete surgical resections with negative tumor margins, chondrosarcomas need a long-term follow-up.

1. Lichtenstein L, Bernstein D. Unusual benign and malignant chondroid tumors of the bone. Cancer 1959; 12:1142-1157.
2. Kundu S, Pal M, Paul Clinicopathologic correlation of chondrosarcoma of mandible with a case report. Contemp Clin Dent 2011; 2:390–393.
3. Chowdhury A, Kalsotra P, Bhagat DR, Sharma P, Katoch P. Chondrosarcoma of the maxilla – recurrent. JK Science 2008; 10:94–6.
4. Bottrill ID, Wood S, Barrett-Lee P, Howard DJ. Mesenchymal chondrosarcoma of the mandible. J Laryngol Otol 1994; 108:785-87.
5. Christensen RE. Mesenchymal chondrosarcoma of the jaws. Oral Surg 1982; 54:197-206.
6. Garrington GE, Collett WK. Chondrosarcoma of the jaw: analysis of 37 cases. J Oral Pathol 1988; 17:12-20.
7. Takahashi K, Sato K, Kanazawa H, Wang XL, Kimura T. Mesenchymal chondrosarcoma of the jaw: report of a case and review of 41 cases in the literature. Head Neck 1993; 15:459-64.
8. Nanda M, Pao ES, Behera KC, Das S, Mohanty L. Fine needle aspiration cytology in malignant bone tumors. Indian J Pathol Microbiol 1994; 37:247-53.
9. Crawford JG, Oda D, Egbert M, Myall R. Mesenchymal chondrosarcoma of the maxilla in a child. J Oral Maxillofac Surg 1995; 53:938-41.
10. Zakkak TB, Flynn TR, Boguslaw B, Adamo AK. Mesenchymal chondrosarcoma of the mandible: Case report and review of literature. J Oral Maxillofac Surg 1998; 56:84-91.