Clear cell lesions in the head and neck region have a wide range of differential diagnosis that includes odontogenic tumors such as ameloblastoma, calcifying epithelial odontogenic tumor, odontogenic carcinoma and salivary gland tumors like mucoepidermoid. Squamous cell carcinoma arising from an odontogenic. The tumor cells were large, with clear cytoplasm, and arranged in irregular sheets. Back to diseases list associated genetic biomarkers overview. Consequently, the name clear cell odontogenic carcinoma was thought to be more appropriate in view of the malignant potential manifested by this neoplastic lesion 2022. Sir, ameloblastic carcinoma ac is a rare malignant odontogenic tumor with very few cases reported in literature so far. Clear cell odontogenic tumor is a rare neoplasm of the jaws that histologically may be confused with metastatic renal cell carcinoma. Clear cell odontogenic carcinoma ccoc, clear cell odontogenic tumor. Clear cell odontogenic carcinoma ccoc is a rare odontogenic tumor of the jaws, histologically characterized by the presence of. Clear cell odontogenic carcinoma ccoc is a rare, lowgrade malignant epithelial neoplasm, occurring in the jawbones, mainly affecting the mandible of elderly patients. Clear cell odontogenic carcinoma tends to be an aggressive tumor with a destructive growth pattern.
Pdf clear cell odontogenic carcinoma of the mandible. Odontogenic cysts, odontogenic tumors, fibroosseous, and. Primary intraosseous squamous cell carcinoma arising from. Pioc must be considered in the differential diagnosis of malignant tumors of odontogenic epithelium, including ameloblastic carcinoma, intraosseous mucoepidermoid carcinoma, clear cell odontogenic carcinoma, and a ma. Hccc is a new and rare lowgrade salivary gland neoplasm which occurs in the primary clear cell carcinoma of minor salivary gland of the soft palate. Here we report a clear cell odontogenic carcinoma of the maxilla in a 55year old male, which was predominantly composed of sheets of polygonal cells with clear cytoplasm, admixed with smaller. However, the clinical and radiographic findings of a mass centered in the mandible supported clear cell odontogenic carcinoma, and breakapart probe confirmed rearrangement of the ewsr1 22q12 locus. The neoplastic cells were cuboidal or polyhedral, clear cell odontogenic carcinoma ccoc is one of the rare1 with centrally placed, rounded nuclei.
Primary clear cell carcinoma of minor salivary gland of. The latter was located at the periphery of the tumor. Ameloblastic carcinoma of the mandible with clear cell. Clear cell odontogenic carcinoma ccoc is described as an exceptional and hard to diagnose malignant tumor which was. A usually aggressive malignant neoplasm arising from toothforming tissues. It is a potentially aggressive tumour, capable of recurring frequently, locally invasive, and with distant metastases. This neoplasm was initially thought to be devoid of malignant potential and classified as benign. Its biological behavior is distinct from that of other tumors, benign and malignant. A 70yearold woman presented with a large painful radiolucent mandibular lesion from the right canine to the left angle area through the midline. Clear cell odontogenic carcinoma ccoc is a rare intraosseous tumor of the jaws. Our goal is to present two new cases, summarize reported cases of clear cell odontogenic carcinoma ccoc, assess potential risk factors for recurrence, and propose definitive surgical and. Misdiagnosis of ameloblastoma in a patient with clear cell odontogenic carcinoma. Oral cavity clear cell odontogenic carcinoma europe pmc. Background clear cell odontogenic carcinoma ccoc, which has been described within the past 2 decades, is a rare odontogenic tumor that tends to occur in the mandible of older adults, with a predilection for women.
Considered benign by who of 1992, but due to its high potential for regional spread and distant metastases, it was reclassified as malignant in 2005. Clear cell odontogenic carcinoma american journal of. Misdiagnosis of ameloblastoma in a patient with clear cell. Odontogenic neoplasms of predominately clear cells are unusual. Objectives to report the clinicopathologic findings and followup. Some of the neoplas benign but locally invasive2 odontogenic tumours in oral cav tic clusters showed a peripheral rim of cells. Clear cell odontogenic carcinoma ccoc is an infrequent tumor, which is aggressive by nature among the odontogenic tumors. Clear cell odontogenic carcinoma is a rare malignant odontogenic neoplasm, with a predilection for the anterior segment of the jaws, and presenting basically in women between the fifth and seventh decades of life. Given the limited number of cases reported, ccoc remains a. Clear cell odontogenic carcinoma is a rare odontogenic tumor occurring predominantly in posterior mandible during 5 th 7 th decades with a female predilection. The tumor was surgically excised and has not recurred or. Clear cell odontogenic carcinoma ccoc is often considered as a rare tumor, first described by hansen in 1985. Its occurrence is most frequent in the posterior mandible 1.
Clear cell odontogenic carcinoma ccoc is a rare odontogenic tumor with predominance in the posterior mandible, usually occurring in the fifth to seventh decades of life, and being found more commonly in females. Clear cell odontogenic carcinoma ccoc is a rare odontogenic tumor arising from the anterior region of mandible and has a predilection for females with 43 cases reported in literature. This article describes a case of clear cell odontogenic carcinoma ccoc in a 55yearold woman who presented such a tumor extending from the midline of the mandible to the right first molar. No recurrence observed while reconstructed in favorable state. Initially it was called as clear cell odontogenic tumor or clear cell ameloblastoma considering its locally destructive nature. It is potentially aggressive and capable of multiple local recurrences and locoregional and distant metastases. Maxillary clear cell odontogenic carcinoma a rare entity. Therefore, clear cell odontogenic carcinoma and hyalinizing clear cell carcinoma of salivary gland were favored, both of which have similar immunoprofiles and molecular genetics. Clear cell odontogenic carcinoma archives of pathology. It is a potentially aggressive tumor, capable of frequent recurrences and locoregional and distant metastases.
In contrast, pioc exhibits sheets or islands of malignant epithelial cells, with minimal or absent clear cell component. Tiejun li, shifeng yu, yan gao, and enbo wang 2001 clear cell odontogenic carcinoma. Br premalatha et al clear cell tumors of the head and neck. They represent a diagnostic dilemma, and as a result, treatment strategies are diverse. Pdf odontogenic neoplasms of predominantly clear cells are unusual. Clear cell odontogenic carcinoma ccoc is a rare odontogenic malignancy with a female predilection, typically presenting as swelling in anterior region of mandible. In the head and neck region, clear cell tumors are usually derived from salivary glands, odontogenic tissues, and metastasis. The term clear cell odontogenic tumor was coined by hansen et al. Wide resection of both the mandibular and tongue base lesions was performed, and the pathologic diagnosis was clear cell odontogenic carcinoma. It has been observed that ameloblastomas can give rise to different types of carcinomas and as clear cell differentiation has also been reported in ameloblastomas, difficulty is encountered in both clinical and histological diagnosis of ameloblastic carcinomas. Clear cell odontogenic carcinoma ccoc is a rare odontogenic malignancy with a female predilection, typically presenting as swelling in. Clear cell odontogenic carcinoma is a rare odontogenic tumor occurring predominantly in posterior mandible during 5th7th decades with a female predilection. Tumor recurred within 5 years of its excision and histopathological examination revealed a clear cell odontogenic carcinoma. Subsequently, similar cases showing aggressive behavior, recurrent behavior and metastatic spread were reported and then designated as clear cell odontogenic carcinoma.
Carcinoma arising in the bone is an extremely rare condition. Clear cell odontogenic carcinoma ccoc is a rare intraosseous carcinoma of the jaw described in 1985 for the first time by hansen 1. Clear cell odontogenic carcinoma and clear cell variant of mucoepidermoid carcinoma are rare and aggressive tumors that usually affect individuals in the fifth decade of life and have a. Clear cell odontogenic carcinoma ccoc is a rare odontogenic tumor of the jaws, histologically characterized by the presence of agglomerates of cells with eosinophilic cytoplasm. Our goal is to present two new cases, summarize reported cases of clear cell odontogenic carcinoma ccoc, assess potential risk factors for recurrence, and propose definitive surgical and therapeutic strategies. Therefore, a link between these tumors seems plausible. C440c449 88253 myofibroblastic sarcoma c470c476,c478c479 88253 myofibroblastic sarcoma 95423 epithelioid malignant peripheral nerve sheath tumor c480c482,c488 87143 pecoma, malignant 88253 myofibroblastic sarcoma c490c496,c498c499. It is characterized by the presence of malignant epithelial cells with clear. Clear cell odontogenic carcinoma is composed of irregular sheets of neoplastic epithelial cells immersed in a richly cellular, collagenous stroma. Clear cell odontogenic carcinoma is a rare odontogenic tumor occurring in the anterior region of the mandible in 5th7th decades and shows a. Odontogenic ghost cell carcinoma displays in addition to a malignant epithelial component the classic features of calcifying odontogenic cyst or tumor, including the pre. Differential diagnosis would include clear cell variant of calcifying epithelial odontogenic tumor, central mucoepidermoid carcinoma, metastatic renal cell carcinoma, and poorly fixed.
The world health organization has classified clear cell odontogenic tumor among benign tumors, but it is now recognized as a more sinister lesion, and current opinion is that it should be designated as a carcinoma. Clear cell odontogenic carcinoma was first described by hansen et al. Mandibular clear cell odontogenic carcinoma ik jae kwon1, soung min kim1,2, emmanuel kofi amponsah2, hoon myoung1, jong ho lee1 and suk keun lee3 abstract background. Clear cell odontogenic carcinoma ccoc is a rare intraosseous carcinoma of the jaw.
A 26yearold man presented with a swelling in the right side of face and ct scan revealed a destructive tumor in the right maxilla. Ccoc was classified as a malignant neoplasm of odontogenic origin by the who in 2005 as it exhibits an aggressive growth pattern, local recurrence, and tendency of distant metastasis. We report a case of ccoc in the mandible of a 45 year old female with lymph node metastasis. Clear cell odontogenic carcinomaa rare case report. The majority of cases represent well differentiated keratinizing carcinoma 6,28. Ghost cell odontogenic carcinoma gcoc was first described in detail as a malignant focus within a calcifying odontogenic cyst coc by ikemura et al. Genetic profile of clear cell odontogenic carcinoma. We report a case of ccoc in the maxillary arch of a 66yearold woman.
It is characterized by aggressive growth, recurrences. Clear cell odontogenic carcinoma report of two cases and. In addition to hyalinizing clear cell carcinoma of the salivary gland, it is one of the epithelial neoplasms known to harbor an ewsr1atf1 fusion. In the english literature, 19 cases of clear cell odontogenic carcinoma, clear cell odontogenic tumor, and clear cell ameloblastoma have been reported. Ccoc is a rare neoplasm with only few cases reported in the literature. The tumor was surgically excised and has not recurred or metastasized 6 months after surgery. Formerly known as clear cell odontogenic tumor, a locally aggressive benign tumor in the who classification of 1992, it was renamed ccoc and classified among malignant tumors after the revision of this classification in 2005. The rarity of this tumor, occurrence in maxilla and young age of the patient are some of the rare features which need. Clear cell odontogenic carcinoma ccoc is a rare aggressive jaw tumor with female predilection that was first described by hansen et al in 1985. However, the clinical and radiographic findings of a mass centered in the mandible supported clear cell odontogenic carcinoma, and breakapart probe confirmed rearrangement of the ewsr1 22q12 locus in 93. It more often affects older females and more frequently occurs in the mandible. Pathogenesis and nomenclature of odontogenic carcinomas.
Clear cell odontogenic carcinoma is a rare entity that tends to arise from the body of the mandible during the fifth through seventh decades and has a female preponderance. In 1992, who classified ccoc as benign odontogenic tumor however, in the who classification of 2005 it was considered to be a malignant tumor of odontogenic origin due to its aggressive nature, predilection to local recurrence, locoregional lymph node. Clear cell odontogenic carcinoma mimicking a cystic lesion. Postoperative radiation therapy was carried out to obviate the possibility of residual disease.
This article describes a case of clear cell odontogenic carcinoma ccoc in a 55. They represent a diagnostic dilemma, and as a result, treatment strategies are. Clear cell odontogenic carcinoma ccoc is a rare odontogenic tumor which occurs mostly in the mandible. Abstractclear cell odontogenic carcinomas are rare, aggressive tumours, most of which have been reported in the mandible of female patients. Pdf clear cell odontogenic carcinoma ccoc researchgate. A case report k a saleh, mrcs entglasg, nurishmah md. A primary intraosseous squamous cell carcinoma pioscc is defined as a squamous cell carcinoma arising within the jaw bones, which has no initial connection with the oral mucous membrane and develops from remnants of odontogenic epithelium deriving embryologically from ledge or epithelial cell rest of malassez.
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