Intraosseous carcinoma arising from an odontogenic cyst: a case report

Vol. 116 No. 6 December 2013

Intraosseous carcinoma arising from an odontogenic cyst: a case report Cihan Bereket, DDS, PhD,a Burak Bekçiog˘lu, DDS, PhD,b Mehmet Koyuncu, MD,c ˙Ismail S¸ener, DDS, PhD,a Bedri Kandemir, MD,d and Akif Türer, DDS,e Samsun and Ordu, Turkey ONDOKUZ MAYIS UNIVERSITY AND ORDU UNIVERSITY

Carcinomas from an odontogenic cyst are not common, yet when they occur, squamous cell carcinomas are the most often seen. Among these cysts, the malignancy of keratocysts or dentigerous cysts are most likely. In contrast, a malignant transformation of a radicular cyst to an intraosseous carcinoma is extremely rare. In this case report, an intraosseous carcinoma arising from an odontogenic cyst in a 26-year-old male patient is presented. This case report clearly demonstrates the importance of the clinician’s awareness of the malignant potential of apparently innocuous cystic lesions. (Oral Surg Oral Med Oral Pathol Oral Radiol 2013;116:e445-e449)

Malignant changes in the epithelial lining of odontogenic cysts have been described in the literature.1-4 Although the exact number of documented cases is difficult to determine, Gardner5 reviewed all documented cases from 1889 to 1967 and reported 25 acceptable examples of malignant transformation within the epithelial lining of an odontogenic cyst. Müller and Waldron6 reported 81 cases documented in the literature worldwide. The incidence of carcinomas arising from odontogenic cysts was reported to be approximately 1 to 2 per 1000.7 According to the revised World Health Organization classification (1992), malignant ameloblastoma, malignant variants of other odontogenic epithelial tumors, which are primarily intraosseous carcinoma and malignant changes in odontogenic cysts, are all classified as odontogenic carcinomas.8 Some authors reported that the epithelial lining of the odontogenic cyst may transform into an odontogenic neoplasmlike ameloblastoma or an adenomatoid odontogenic tumor.9-11

Assorted odontogenic cysts have been associated with odontogenic carcinomas, including residual cysts, dentigerous cysts, calcifying odontogenic cysts, and lateral periodontal cysts. Residual cysts are the most commonly associated cysts followed by dentigerous cysts.12 Even though odontogenic keratocysts are well known for their aggressive biological potential,13 only 16 cases of malignant transformation have been reported in the literature.14-26 All well-documented case reports of primary intraosseous squamous cell carcinomas arising from odontogenic cysts published between 1938 and 2010 were collected by Bodner et al.27 The tabulation of these 116 cases and the results are shown in Table I. Differential diagnoses of odontogenic cysts and malignant tumors arising in a cyst may be difficult owing to their nonspecific clinical and radiological presentation.28 This report presents a case of an intraosseous carcinoma arising from an odontogenic cyst.

CASE REPORT a

Assistant Professor, Ondokuz Mayis University, Faculty of Dentistry, Department of Oral and Maxillofacial Surgery, Samsun, Turkey. b Assistant Professor, Ordu University, Faculty of Dentistry, Department of Oral and Maxillofacial Surgery, Ordu, Turkey. c Professor, Ondokuz Mayis University, Faculty of Medicine, Department of Otolaryngology, Head and Neck Surgery, Samsun, Turkey. d Professor, Ondokuz Mayis University, Faculty of Medicine, Department of Pathology, Samsun, Turkey. e Research Assistant, Ondokuz Mayis University, Faculty of Dentistry, Department of Oral and Maxillofacial Surgery, Samsun, Turkey. Received for publication Jul 27, 2011; returned for revision Feb 6, 2012; accepted for publication Feb 28, 2012. © 2013 Elsevier Inc. All rights reserved. 2212-4403/$ - see front matter http://dx.doi.org/10.1016/j.oooo.2012.02.029

A 26-year-old male patient was referred to the Ondokuz Mayis University, Faculty of Dentistry, Department of Oral and Maxillofacial Surgery, after suffering from a painful swelling in his upper jaw for 3 years. According to the patient, a yellowish leakage and pain occasionally bothered him. He had no history of tobacco or alcohol use and no systemic disease. A clinical examination revealed the expansion in the vestibular and palatal region of the anterior maxilla, predominantly on the right side (Figure 1). A small amount of brownish liquid aspirated in a fine needle biopsy (Figure 2). Panoramic radiography and computerized tomography revealed a well-demarcated, radiolucent cystlike lesion between the right second premolar and left first premolar that involved the roots of the neighboring teeth (Figures 3 and 4). The vitalometric analyses showed late responses in related teeth.

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Table I. Tabulation of the reported carcinomas arising from odontogenic cysts27

Decade of life 0-2 3-5 Sex Male Female Site Mandible Maxilla Treatment modality Surgery alone Surgery ⫹ radiotherapy Surgery ⫹ radiotherapy ⫹ chemotherapy Surgery ⫹ chemotherapy Radiotherapy alone Neck dissection Overall survival, y 2 5

No. of cases

Percentage of cases, %

2 33

2 28

80 36

68 32

92 24

79 21

53 44 7

46 38 6

7 4 59

6 4 51

58 36

62 38

Fig. 2. Aspirated specimen from the lesion.

Fig. 3. Panoramic radiography of the patient.

Fig. 1. Intraoral view of the patient.

Treatment with marsupialization was considered owing to the dimensions of the lesion, but because of the inconclusive biopsy, the thickening of the cyst walls in the cavity, and infiltration of the lesion into the left nose wing, total enucleation of the cyst was the decided treatment. All anterior teeth, including right and left first premolars, received root canal treatments before surgery. The enucleation was performed under local anesthesia without any complications (Figure 5). The pathologic examination revealed squamous cell carcinoma along with keratinized odontogenic cyst epithelium (Figure 6). Two weeks after the first surgery, a partial maxillectomy, including all the maxilla for the upper left second and third molars, was performed under general anesthesia (Figure 7). Neck dissection was not performed. A maxillary surgical stent was made for feeding and speaking (Figure 8). Follow-up visits are continuing.

Fig. 4. Axial CT view of the lesion.

DISCUSSION Jaw bones are the only bones that contain epithelial tissues and that are capable of developing connective tissue tumors as well as epithelial tumors.29 Squamous cell carcinomas of the jaw located completely in the bone are extremely rare. Most of these intraosseous carcinomas, also called odontogenic carcinomas, are thought to arise from the epithelial lining of an odon-

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Fig. 5. Lesion enucleation under local anesthesia. Fig. 8. The view of the surgical stent applied after the maxillectomy.

Fig. 6. Well-differentiated squamous cell carcinoma arising from the epithelium of cystic formation that is covered with keratinized multilayer epithelium. The tumor consists of atypical cells with hyperchromatic nuclei and they have irregularly infiltrated into the wall. (Hematoxylin & eosin stain, magnification ⫻40.)

Fig. 7. The view of the maxilla after a partial maxillectomy.

togenic cyst.30 Primary intraosseous carcinomas are far more common in the mandible than in the maxilla,31 but in our patient, the malignant transformation of the odontogenic cyst occurred in the maxilla.

Although all odontogenic cysts have the potential for malignant transformation, the potential seems higher in the cases of inflammatory cysts. More than 50% of the reported cases have arisen from inflammatory periapical or residual cysts.5,13,32-34 Long-standing chronic inflammation has been suggested as a predisposing factor. Gardner35 and Yu et al.36 proposed that the possibility of malignant change in an odontogenic cyst is very low when there is no inflammation. In our patient, the signs of chronic inflammation were evident. Van der Wal et al.37 suggested that the existence of keratinization in the cyst lining is a risk factor for malignant changes. Browne et al.34 noted that keratinizing odontogenic cysts are more prone to malignant changes than nonkeratinizing types. Anneroth and Hansen38 believed that the most common factor might be an inflammatory reaction with or without a genetic background. Because most reports present only isolated cases, the prognosis is difficult to evaluate. The reported 2-year survival rate lies between 53% and 63%,39,40 whereas the 5-year survival rate is 30% to 40%.41,42 The diagnosis of a primary intraosseous carcinoma arising from an odontogenic cyst is rare, but it is often worth considering as a differential diagnosis of a jaw radiolucency, especially in older patients with long-standing “cystic” lesions in their jaws.30 Radiographically, malignant changes in an odontogenic cyst should be considered if the radiolucent area, usually unilocular, has jagged or irregular margins with indentations and indistinct borders, suggestive of invasion and destruction by a tumor, or if serial occlusal radiographs over a short interval show rapid enlargement of the radiolucent area,43 especially if there is erosion of the buccal, labial, and lingual plates.20 In our case, the erosion was seen in the anterior buccal cortex of the maxilla. Panoramic radiography can be useful in cases of gross diseases, but it has limited value in

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Fig. 9. The algorithm for decision making in odontogenic cyst treatment.

evaluating margins and extension and invasion of the tumor mass because of the superimposition of bony structures and the fact that it does not show soft tissue masses well.44 In our patient, it failed to demonstrate the aggressive behavior of the tumor. Surgical treatments for odontogenic cysts include total enucleation of small lesions, marsupialization for decompression of larger cysts, or a combination of these techniques. When surgical intervention becomes necessary, the clinician must decide whether to enucleate the lesion completely or to try “decompression” first.45,46 Enucleation is the preferred treatment for odontogenic cysts; however, when the lesion is large, marsupialization can be performed owing to the risk of fracture or harming any important organ or tissue during the removal of the lesion by enucleation.13 If marsupialization with decompression is attempted first, the size of the lesion will be reduced, which will make it less difficult to remove, with less risk of damage to the neighboring teeth and vital structures.47 For the present case, marsupialization was the initial treatment planned because of the large size of the lesion. However, the surgical literature clearly indicated enucleation of the cyst as the preferred option because marsupialization carries the risk that any cystic cells left behind may be or could become malignant5,48 and the relatively long time needed for the treatment would lead to expanding or spreading of the malignancy.

Thickening regions at the wall of the lesion and the lack of a cystic liquidlike material after the aspiration biopsy indicated a probable malignancy. A biopsy is recommended as well as removing the entire cyst wall, as malignant changes in the epithelial lining might not be seen in all parts of the lesion.30 A treatment algorithm is presented in Figure 9. In our case, a pathologic examination was performed on the cyst wall specimen and a malignant transformation was detected. After the detection of this malignancy, a maxillectomy was unavoidable. After the maxillectomy operation, an immediate temporary surgical stent helped the patient in feeding and speaking. A permanent obturator-type denture will be made after the soft tissue heals and related oral and nasal mucosas are remodeled. The patient was stable 2 months after the operation. To date, neither a recurrence nor a metastasis has occurred and follow-up visits are continuing. REFERENCES 1. Suei Y, Tanimoto K, Taguchi A, Wada T. Primary intraosseous carcinoma: review of the literature and diagnostic criteria. J Oral Maxillofac Surg 1994;52:580-3. 2. Bridgeman A, Wiesenfeld D, Buchanan M, Slavin J, Costello BA. Primary intraosseous carcinoma of the anterior maxilla. Report of a new case. Int J Oral Maxillofac Surg 1996;25:279-81. 3. Dayal PK, Rawal YB. Primary intraosseous carcinoma of the jaws originating in odontogenic cysts. Indian J Cancer 1997;34:6-11.

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