Clear cell odontogenic carcinoma: A diagnostic and therapeutic dilemma

Institute of Pathology-ICMR, Safdarjung Hospital Campus, New Delhi, India.
World Journal of Surgical Oncology (Impact Factor: 1.41). 02/2006; 4(1):91. DOI: 10.1186/1477-7819-4-91
Source: PubMed


Clear cell odontogenic carcinoma is a rare odontogenic tumor occurring in the anterior region of the mandible in 5th-7th decades and shows a female preponderance. It is potentially aggressive, capable of frequent recurrences and loco-regional and distant metastases.
A 45-year-old woman presented with a radiolucent left mandibular swelling associated with loss of teeth. Left cervical lymph nodes were enlarged on palpation. The patient underwent resection of the tumor but consequent to resected margins being positive for tumor cells underwent left hemimandibulectomy with ipsilateral functional neck dissection and was free of recurrence at 8 months follow-up.
Clear cell odontogenic carcinoma should be considered in the differential diagnosis of jaw tumors with conspicuous clear cell component. Curettage or conservative resection inevitably results in recurrences and/or metastasis and more radical resection is warranted in these tumors, especially when they are large and show soft tissue invasion.


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    • "Such tumors include calcifying epithelial odontogenic tumor and clear cell ameloblastoma. While the former is identified by the presence of psammomatous calcifications and amyloid deposits, the latter may be difficult to distinguish from CCOC [13]. In fact, some authors thought that clear cell ameloblastomas and CCOCs might represent a clinicopathological continuum of a single neoplastic entity [14]. "
    [Show abstract] [Hide abstract] ABSTRACT: Background: Clear cell odontogenic carcinoma (CCOC) is a rare intraosseous carcinoma of the jaw; only 81 cases have been reported in the English literatures. Case presentation: We reported an additional case and reviewed the existing literature. A 70-year-old woman presented with a large painful radiolucent mandibular lesion from the right canine to the left angle area through the midline. No metastatic lymph nodes or distant metastases were detected. She underwent wide surgical resection and reconstruction with a composite fibula free flap. She had no recurrence or metastasis after 18 months. Conclusion: CCOC occurs predominantly in women in their 50s-70s in the mandible. Painless swelling is the most common symptom, followed by pain, teeth loosening, and paresthesia. CCOC has a good prognosis after surgery. In large mandibular CCOC, wide resection and composite fibula free flap reconstruction is the treatment of choice.
    Full-text · Article · Dec 2015 · World Journal of Surgical Oncology
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    • "A wide resection with at least 1 cm of a tumor-free margin is recommended and when there is evidence broad soft tissue invasion, palpable neck lymph node, perineural invasion or tumor removal without free margins, adjuvant neck dissection and/or radiotherapy should be considered10,12,13. However, except for squamous cell carcinoma and high-grade central mucoepidermoid carcinoma, neck dissection is rarely required for maxillary carcinomas without nodal metastases12,13,14. "
    [Show abstract] [Hide abstract] ABSTRACT: Clear cell odontogenic carcinoma (CCOC) is a rare jaw tumor that was classified as a malignant tumor of odontogenic origin in 2005 by the World Health Organization because of its aggressive and destructive growth capacity and metastasis to the lungs and lymph nodes. We report a case of a 66-year-old female who had swelling, incision and drainage history and a well-defined unicystic radiolucent lesion that was comparable to a cystic lesion. At first, the patient received decompression, and the lesion size decreased. Three months after decompression, cyst enucleation was performed. The pathologic result indicated that the lesion was CCOC. In this report we emphasize that patients with painful cystic lesions in addition to jaw enlargement and loosening teeth should be considered for the possibility of malignancy.
    Full-text · Article · Aug 2014
    • "Glycogen storage is quite common in these tumors displaying PAS positivity which is diastase sensitive.[921–2325] The reports on immunohistochemical findings of CCOC have been inconsistent. "
    [Show abstract] [Hide abstract] ABSTRACT: Clear cell odontogenic carcinoma is a rare odontogenic tumor occurring predominantly in posterior mandible during 5(th)-7(th) decades with a female predilection. It is a potentially aggressive tumor, capable of frequent recurrences and loco-regional and distant metastases. Till date, only 73 cases have been reported in the literature. Current case is of a 55-year-old woman with tumor mass extending from canine to molar region on the left maxillary arch. Being locally aggressive tumor with the capacity to metastasize, it demands to be distinguished from other primary and metastatic clear cell tumors of the oral and maxillofacial region. A brief compilation of the reported cases is being attempted in the current article to better understand the behavior of the tumor.
    No preview · Article · Apr 2013 · Journal of Oral and Maxillofacial Pathology
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