Odontogenic Carcinosarcoma: Case Report and Literature Review

Odontogenic Carcinosarcoma: Case Report and Literature Review

J Oral Maxillofac Surg 69:1501-1507, 2011 Odontogenic Carcinosarcoma: Case Report and Literature Review Ruwadzano Chikosi, DDS,* Norberto Segall, DDS...

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J Oral Maxillofac Surg 69:1501-1507, 2011

Odontogenic Carcinosarcoma: Case Report and Literature Review Ruwadzano Chikosi, DDS,* Norberto Segall, DDS,† Pereira Augusto, MD,‡ and Paul Freedman, DDS§ We report the case of an odontogenic carcinosarcoma in a 9-year-old girl. The tumor destroyed both lingual and buccal cortices of the right mandible. A mandibulectomy was performed, and the specimen was a fleshy fluctuant mass. Microscopic evaluation showed a biphasic appearance with malignant features of both the epithelial and fibroblastic components. The patient was never free of the disease from the point of surgery until she died 2 years 3 months later. This is the fifth case of odontogenic carcinosarcoma to be reported in the literature and the first case to be reported in a child. Odontogenic carcinosarcoma is an extremely rare tumor thought to arise from odontogenic rests. To date, only a few cases have been reported. It is more common in the mandible. The patients’ ages range from 19 to 63 years, and there is no gender predilection. It is a true mixed tumor showing malignant cytology of both the epithelial and mesenchymal components. Even with a few cases reported, the clinical behavior of the tumor is considered aggressive. Many are thought to arise from pre-existing lesions such as ameloblastoma, ameloblastic fibroma, and ameloblastic fibrosarcoma. Treatment is primarily surgical resection, but with the increased frequency to metastasize to the lungs and regional lymph nodes, radiotherapy and chemotherapy may be warranted.

of the face and facial asymmetry (Fig 1). A physical examination of the patient showed a large, purple, fluctuant mass stretching from the second premolar to the retromolar pad area (Fig 2). She had slight pain on palpation, malaise, and numbness of the lip on the affected side from the previous surgery. She had some difficulty swallowing and was receiving a soft diet. On presentation, opening and closing motions were normal and no fever or lymphadenopathy was noted. The patient’s dental history showed that in November 2005 she presented to her general dentist, and panoramic images showed a radiolucent lesion extending from the area distal to the first molar to the retromolar pad area (Fig 3). An incisional biopsy was performed, and the diagnosis was

Report of Case A 9-year-old female patient presented to a dental office in Venezuela in January 2006 with a swelling of the right side *Resident, Section of Oral Pathology, New York Hospital Queens, Flushing, NY. †Director of Oral and Maxillofacial Pathology, New York Hospital Queens, Flushing, NY. ‡Oral and Maxillofacial Surgeon, Dental Department of Hospital de Clinicas, Caracas, Venezuela. §Chief Director of Pediatric Oncology, J.M. de los Rios Hospital, Caracas, Venezuela. Address correspondence and reprint requests to Dr Chikosi: Section of Oral Pathology, New York Hospital Queens, 56-31 141st St, Flushing, NY 11355; e-mail: [email protected] © 2011 American Association of Oral and Maxillofacial Surgeons

0278-2391/11/6905-0052$36.00/0 doi:10.1016/j.joms.2010.05.071

FIGURE 1. Facial asymmetry and swollen right side of face (Jan 2006). Chikosi et al. Odontogenic Carcinosarcoma. J Oral Maxillofac Surg 2011.

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ODONTOGENIC CARCINOSARCOMA with clear margins was performed with jaw reconstruction. The surgery was done based on the 2 prior diagnoses. The specimen was then sent to a pathologist in Venezuela, who reported a diagnosis of odontogenic carcinoma. In March 2006 a consultation was requested with the Oral Pathology Laboratory Inc, located at New York Hospital Queens, yielding a diagnosis of odontogenic carcinosarcoma. The differential diagnosis included recurrent ameloblastoma, ameloblastic fibrosarcoma, ameloblastic carcinoma, and other related odontogenic tumors.

Pathologic Findings FIGURE 2. Large lobulated purplish soft tissue mass of right mandible (Jan 2006). Chikosi et al. Odontogenic Carcinosarcoma. J Oral Maxillofac Surg 2011.

ameloblastoma. The patient was treated with curettage. However, in December 2005 she had a relapse (Fig 4), and another biopsy with curettage was performed. The diagnosis was recurrent ameloblastoma. She had no other known medical conditions. On the January 2006 visit, imaging studies showed a large radiolucent lesion with cortical perforation (Figs 5, 6). Several computed tomography scans, magnetic resonance imaging scans, and blood tests were done to rule out metastatic disease. Three weeks later, a partial mandibulectomy

A gross examination of the specimen showed a 7.0-cm multinodular mass of the right posterior mandible with adjacent perforated cortices. The specimen had a fleshy fluctuant consistency (Fig 7). Microscopic examination of hematoxylin-eosin slides showed a tumor composed of malignant features of both the epithelial and mesenchymal components (Fig 8). There were large ameloblastic epithelial islands with edematous stellate reticulum–like areas. The islands were composed of large pleomorphic cells and nuclei, hyperchromatic nuclei, and atypical mitotic figures (Figs 9, 10). The mesenchymal component also exhibited the same malignant features (Fig 11). Immunohistochemical stains helped to confirm the diagnosis with the epithelial component

FIGURE 3. Radiograph showing radiolucent lesion distal to first molar (Nov 2005). Chikosi et al. Odontogenic Carcinosarcoma. J Oral Maxillofac Surg 2011.

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FIGURE 4. Radiograph showing larger radiolucent lesion without defined borders extending to retromolar pad area (Dec 2005). Chikosi et al. Odontogenic Carcinosarcoma. J Oral Maxillofac Surg 2011.

staining intensely positive with AE1/AE3 (Fig 12) and the mesenchymal component intensely and diffusively positive with vimentin (Fig 13).

Treatment and Follow-Up In January 2006 surgery was done based on the 2 prior biopsies. A partial mandibulectomy with careful preservation of the condyle and the angle of the mandible was performed. On histopathologic examination, all margins were free of tumor, and immediate reconstruction with 2.4-mm plates was done to stabilize the mandible (Figs 14, 15). However, in April 2006 the patient had 2 more interventions because of relapses. A month later, chemotherapy was initiated with carboplatin, ifosfamide, and etoposide with a partial response of approximately 50%. However, the tumor progressed rapidly, and the protocol was changed to gemcitabine and docetaxel with no significant tumor response. In view of the non-chemosensitivity of the tumor, radiation therapy was initiated in September 2006. A dose of 3,575 cGy to the primary tumor site followed by 2,600 cGy to the lower neck in conjunction with chemotherapy was begun. With this treatment, only a partial response was achieved. A Morganella morga-

nii infection and severe oral mucositis developed because of the radiotherapy. A gastrostomy was performed to ensure adequate nutrition, but unfortunately, the patient died in April 2008 of complications from the disease.

Discussion Ameloblastic or odontogenic carcinosarcomas are believed to arise from related neoplasms such as ameloblastoma, ameloblastic fibroma, and ameloblastic fibrosarcoma. Their transformation to malignant tumors is not fully understood, but this usually occurs after multiple recurrences and surgical treatments without adjuvant therapy. These tumors, like all other odontogenic tumors, arise from epithelial rests that are remnants of the embryologic process of tooth production. The 4 previously published cases all report the presence of a pre-existing lesion.1-4 Tanaka et al1 reported a case in which the ameloblastoma transformed to a carcinosarcoma after multiple resections and radiation therapy. The case reported by Slama et al2 was originally called ameloblastoma, but after metastasis to the lymph nodes, it was reclassified as a carcinosarcoma. The patient of Kunkel et al3 was initially diagnosed

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FIGURE 5. Axial computed tomography scan showing expansile radiolucent lesion of mandible (Jan 2006). Chikosi et al. Odontogenic Carcinosarcoma. J Oral Maxillofac Surg 2011.

with fibrosarcoma, whereas the case of Delair et al4 had an ameloblastic fibroma. In our case, even though the lesion was initially diagnosed as ameloblastoma, the clinical course and biological behavior warranted a thorough review. The multiple recurrences within a short amount of time, distant metastasis, and failure to control the disease with chemotherapy and radiotherapy speak to its malignant nature. Therefore there is a great chance that the initial lesion was a malignant odontogenic tumor. Given the rarity of odontogenic carcinosarcoma, it is difficult to categorize, but it is evident that it is an aggressive tumor with the potential to metastasize, which distinguishes it from other related neoplasms. Of all the published cases, 1 case metastasized to the lungs and 2 cases to the regional lymph nodes.2,3 Only one case by Delair et al4 and Tanaka et al1 had no recurrences or metastasis after a 2-year follow-up. When adequately treated, the recurrence rates for ameloblastomas and ameloblastic fibromas are very low. Occasionally, if benign odontogenic lesions are longstanding, malignant transformation may occur. In the case of ameloblastic fibrosarcomas, their malignant behavior is different from that of odontogenic carcinosarcomas. Death is usually the result of uncontrolled

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FIGURE 6. Axial computed tomography scan showing perforation of lingual cortex (Jan 2006). Chikosi et al. Odontogenic Carcinosarcoma. J Oral Maxillofac Surg 2011.

FIGURE 7. Resected specimen showing 7.0-cm fluctuant gelatinous mass of right mandible (Mar 2006). Chikosi et al. Odontogenic Carcinosarcoma. J Oral Maxillofac Surg 2011.

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FIGURE 8. Malignant epithelial and mesenchymal component (hematoxylin-eosin stain, original magnification ⫻10). Chikosi et al. Odontogenic Carcinosarcoma. J Oral Maxillofac Surg 2011.

FIGURE 9. Malignant epithelial component showing large pleomorphic cells with enlarged nuclei (hematoxylin-eosin stain, original magnification ⫻40).

FIGURE 10. Edematous stellate reticulum with large pleomorphic cells and hyperchromatic nuclei (hematoxylin-eosin stain, original magnification ⫻40).

Chikosi et al. Odontogenic Carcinosarcoma. J Oral Maxillofac Surg 2011.

Chikosi et al. Odontogenic Carcinosarcoma. J Oral Maxillofac Surg 2011.

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FIGURE 11. Mesenchymal component showing pleomorphic cells with very hyperchromatic nuclei (hematoxylin-eosin stain, original magnification ⫻20).

FIGURE 13. Mesenchymal component showing diffuse and intense positivity with vimentin (immunohistochemical stain, original magnification ⫻10).

Chikosi et al. Odontogenic Carcinosarcoma. J Oral Maxillofac Surg 2011.

Chikosi et al. Odontogenic Carcinosarcoma. J Oral Maxillofac Surg 2011.

FIGURE 12. Epithelial island showing intense positivity with cytokeratin AE1/AE3 (immunohistochemical stain, original magnification ⫻10). Chikosi et al. Odontogenic Carcinosarcoma. J Oral Maxillofac Surg 2011.

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FIGURE 15. Healing soft tissue after surgery and reconstruction. FIGURE 14. Mandibulectomy after diagnosis of odontogenic carcinosarcoma.

Chikosi et al. Odontogenic Carcinosarcoma. J Oral Maxillofac Surg 2011.

Chikosi et al. Odontogenic Carcinosarcoma. J Oral Maxillofac Surg 2011.

References

local recurrences, and metastasis is very rare. The malignant changes are seen only in the mesenchymal portion. In odontogenic carcinosarcoma, both the epithelial and mesenchymal portions show abundant large cells, hyperchromasia of the nuclei, and atypical mitotic figures. The few cases reported suggest that these tumors have a high propensity to metastasize. More cases are needed to fully understand the behavior of the neoplasm and how to best treat it.

1. Tanaka T, Ohkibo T, Fujitsuka H: Malignant mixed tumor (malignant ameloblastoma and fibrosarcoma of the maxilla). Arch Pathol Lab Med 115:84, 1991 2. Slama A, Yacoubi T, Khochatali H, et al: Mandibular odontogenic carcinosarcoma: A case report. Rev Stomatol Chir Maxillofac 103:124, 2002 3. Kunkel M, Ghalibafian M, Radner H, et al: Ameloblastic fibrosarcoma or odontogenic carcinosarcoma: A matter of classification? Oral Oncol 40:444, 2004 4. Delair D, Bejarano P, Peleg M, et al: Ameloblastoma carcinosarcoma of the mandible arising in ameloblastic fibroma: A case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 103:516, 2007