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BACKGROUND: Clear cell odontogenic carcinoma (CCOC) is an odontogenic carcinoma characterized by sheets and islands of vacuolated and clear cells. The diagnosis of atypical CCOC can pose a challenge when tumor cells deviate from their characteristic clear morphology, even with the aid of genetic profiling for CCOC identification. CASE PRESENTATION: In this manuscript, we detailed the inaugural instance of a recurrently recurring clear cell odontogenic carcinoma (CCOC) with pronounced squamous differentiation in a 64-year-old male. The primary tumor in this individual initially displayed a biphasic clear cell phenotype. However, subsequent to the third recurrence, the clear tumor cells were entirely supplanted by epidermoid cells characterized by eosinophilic cytoplasm, vesicular chromatin, and prominent nucleoli. Notable aggressive attributes such as necrosis, conspicuous cytological malignancy, perineural dissemination, and vascular invasion were noted. Additionally, the tumor progressed to manifest lung metastases. The tumor cells exhibited positive immunoreactivity for AE1/AE3, KRT19, Pan-CK, EMA, P40, P63, CK34ßE12, and P53, while they tested negative for CK35ßH11, KRT7, S-100, and neuroendocrine markers. The Ki-67 proliferation index was calculated at an average of 15%. Furthermore, FISH analysis unveiled the presence of the EWSR1::ATF1 gene fusion. CONCLUSIONS: This case illustrated a rare and aggressive case of CCOC characterized by significant squamous differentiation upon recurrence of the tumor.

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In the 2022, World Health Organisation classification of odontogenic tumours, the clear cell odontogenic carcinoma is designated as a malignant odontogenic tumour with high recurrence and aggressive behaviour. Deceptive behaviour in the context of a wide range of differentials presents a significant diagnostic problem. It is the fifth most commom type of malignant odontogenic tumor. A systematic assessment of published cases, case series, and retrospective investigations of diagnostic significance of EWSR1 gene in clear cell odontogenic carcinoma is presented to determine trends in presentation, diagnostic characteristics, treatment, and patient outcome. To locate papers reporting clear cell odontogenic carcinoma and EWSR1, extensive database searches were carried out. Demographics, tumour location, immunohistochemical and molecular tests, treatment, follow-up, and recurrence were the variables. 34 cases were detected; 52.9% (n = 18) of the cases were females. The average age was 62.5 years, with a range of 43-82 years. The average size ranged from 3.4 to 8 cm. The mandibular body was the most common location, followed by the maxilla. Maximum immunohistochemistry positivity revealed by CK 19, CKAE1/3, EMA and p63. Most common gene fusion detected was EWSR1-ATF1 in 62.4% of cases contributing to its diagnostic attributes. Surgical treatment was used in 97% of cases. The average follow-up period was 30.3 months, and recurrence was reported in 52.4% of the cases. CCOC can metastasize, and the prognosis is fair. This is first systematic review, where we have attempted to consolidate the mutational expression of EWSR1 in Clear cell odontogenic carcinoma. It is difficult to identify from other clear cell tumours of the head and neck region. It is crucial to distinguish it from other clear cell lesions because of its aggressiveness.

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OBJECTIVE: To review tumors identified as "clear cell sarcoma" in order to determine similarities to the rare EWS fusion positive jaw and salivary gland tumors clear cell odontogenic carcinoma (CCOC) and clear cell carcinoma of the salivary gland (CCC). METHODS: PubMed was used to collect all reports of clear cell sarcoma (CCS). Search parameters were "clear cell sarcoma" and "CCS." References in the publications were screened and cross-referenced. Data extracted included demographic characteristics, presenting signs and symptoms, radiographic findings, histological and immunohistochemical features and known molecular/genetic aberrations. RESULTS: Clear cell sarcoma has several similarities to CCOC and CCC. All three tumor types have similar histologic appearances including the presence of clear cells, as well as similar genetic profiles in that all harbor an EWSR1-CREB family fusions. Additionally, these tumors appear in soft tissue as well as bone, and can have a prolonged clinical course. CCS can appear anywhere in the body, including the head and neck region. All three tumors appear to have a predilection to women, although CCS may have a slight younger age of onset as compared to CCOC and CCC (3rd vs 5th decade of life, respectively). CONCLUSION: Gaining a better understanding of the similarities and differences between these three tumors may lead to a better understanding of each one.

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Clear cell odontogenic carcinoma (CCOC) is a rare epithelial malignant odontogenic tumor of the jaw with a distinct histology and deceptive behavior. As the clinical presentation is often misleading, the contribution of biopsy, molecular biology, immunohistochemistry and cytogenetics are essential in the diagnostic process. This article describes the presentation of an aymptomatic, painless swelling in the lower jaw of a 63 yrs old female, who was initially diagnosed as an ameloblastoma, taken up for segmental resection, subsequently histopathologically diagnosed as a case of clear cell odontogenic carcinoma and later managed with chemotherapy. Current management protocols and presentation of CCOC have also been reviewed.

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Clear cell odontogenic carcinoma (CCOC) is a rare odontogenic neoplasm with high risk of recurrence and aggressive behaviour. Presence of clear cells in head and neck is hallmark, but not pathognomic for the entity. Deceptive behaviour admist wide range of differentials, poses an immense diagnostic challenge. An incisional biopsy with appropriate immunohistochemistry is prudent for diagnosis. We herein report atypical case of CCOC mimicking squamous cell carcinoma, in a tertiary cancer center in North Eastern part of India.

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Clear cell odontogenic carcinoma is a rare malignancy, which is vary in behavior from indolent tumor to cases with frequent recurrence and rare metastasis. In this manuscript, we report a case of clear cell odontogenic carcinoma of right lower mandible with cervical lymph node metastasis in a 50-year-old female. The tumor recurred and metastasized to vertebra and pleura after adjuvant radiation and chemotherapy, and the patient died of the disease 29 months after diagnosis. Pathologically, the tumor was composed of epithelial nests dominated by clear cells with ameloblastoma-like pattern and biphasic pattern. The tumor cells showed strong positive for AE1/AE3, KRT19, KRT5/6, P63, focal positive for KRT7, and weak positive for MUC1. Molecularly, EWSR1::ATF1 gene fusion was identified. To our knowledge, this report describes the first case of metastasis of clear cell odontogenic carcinoma involving regional lymph nodes and distant pleural and vertebral areas, demonstrating an aggressive clinical course of clear cell odontogenic carcinoma.

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BACKGROUND: Homeobox genes play crucial roles in tooth morphogenesis and development and thus mutations in homeobox genes cause developmental disorders such as odontogenic lesions. The aim of this scoping review is to identify and compile available data from the literatures on the topic of homeobox gene expression in odontogenic lesions. METHOD: An electronic search to collate all the information on studies on homeobox gene expression in odontogenic lesions was carried out in four databases (PubMed, EBSCO host, Web of Science and Cochrane Library) with selected keywords. All papers which reported expression of homeobox genes in odontogenic lesions were considered. RESULTS: A total of eleven (11) papers describing expression of homeobox genes in odontogenic lesions were identified. Methods of studies included next generation sequencing, microarray analysis, RT-PCR, Western blotting, in situ hybridization, and immunohistochemistry. The homeobox reported in odontogenic lesions includes LHX8 and DLX3 in odontoma; PITX2, MSX1, MSX2, DLX, DLX2, DLX3, DLX4, DLX5, DLX6, ISL1, OCT4 and HOX C in ameloblastoma; OCT4 in adenomatoid odontogenic tumour; PITX2 and MSX2 in primordial odontogenic tumour; PAX9 and BARX1 in odontogenic keratocyst; PITX2, ZEB1 and MEIS2 in ameloblastic carcinoma while there is absence of DLX2, DLX3 and MSX2 in clear cell odontogenic carcinoma. CONCLUSIONS: This paper summarized and reviews the possible link between homeobox gene expression in odontogenic lesions. Based on the current available data, there are insufficient evidence to support any definite role of homeobox gene in odontogenic lesions.

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Both clear cell odontogenic carcinoma (CCOC) and sclerosing odontogenic carcinoma (SOC) are rare odontogenic malignancies. Here, we report a case of maxillary CCOC whose clinical and histologic features resembled those of SOC. Radiologically, the tumor presented as an ill-defined, expansile radiolucency with local bone destruction. Histologically, the tumor was comprised of thin cords or strands of odontogenic epithelium permeating through a sclerosed fibrous stroma with occasional clear cell foci. It damaged the cortical plates and invaded the adjacent soft tissue. Immunohistochemical expression of Pancytokeratin, Cytokeratin 19, p63, Cytokeratin 5/6, and Cytokeratin 14, as well as focal expression of Cytokeratin 7, demonstrated the epithelial nature of the tumor. Alcian Blue Periodic acid Schiff staining revealed a lack of intracellular mucin. Fluorescence in situ hybridization analysis revealed Ewing sarcoma RNA binding protein 1 and activating transcription factor 1 gene translocation, further confirming the diagnosis of CCOC. Lastly, we contextualized the genetic analysis of our case to that of CCOC in the literature.

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Clear cell odontogenic carcinoma (CCOC) is a rare, aggressive epithelial neoplasm of the jaw first described in 1985 by Hansen and classified as an odontogenic malignant tumor in the 2005 WHO classification. To date, only 117 cases have been reported in the literature written in English. In this paper, we report the atypical presentation of a pericoronal localized tumor in the right mandibular wisdom tooth discovered during a routine radiologic examination. The patient, a 44-year-old healthy female, was referred by her general dental practitioner for examination of temporomandibular dysfunction and recurrent myofascial pain. Anamnesis and clinical examination did not suggest tumoral disease. The osteolytic lesion was removed, and histological examination revealed a clear cell odontogenic tumor (CCOT) of the right posterior lower jaw. Segmental lower jaw resection was performed, and a free iliac crest bone transplant was fixed with mandibular reconstruction plate. No cervical neck dissection was needed. A five-year follow-up examination shows excellent local and systemic recovery and no sign of tumor recurrence. The patient still suffers from bruxism and myofascial related pain, which are treated conservatively with a Michigan splint and physiotherapy. Based on this additional case, we review the literature and discuss the challenging diagnostic aspects, the unusual clinical presentation, and the treatment of CCOC.

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Clear cell Odontogenic Carcinoma (CCOC) is an uncommon malignant odontogenic tumor (MOT). It is the fifth most common MOT. A systematic review is presented of reported cases, case series and retrospective studies of CCOC, to determine trends in presentation, diagnostic features, treatment, and patient outcome. Searches of detailed databases were carried out to identify papers reporting CCOC. The variables were demographics, patient symptoms, tumor location, histopathological findings, immunohistochemical studies, treatment, follow-up, and recurrence. 117 cases were identified; CCOC was most frequently seen in mature females 65% (n = 76). The total average age was 55.4 with a range from 17 to 89 years, for females 56.4 and males 53.6 years. The mean size was 3.41 cm. The most common location was in the mandibular body 36.2% (n = 42), followed by the anterior mandible 23.3% (n = 27). The most common clinical presentation was a swelling 80.4% (n = 74), and the main symptom was pain 41.3% (n = 31), followed by painless lesion 24% (n = 18). The most common Immunohistochemistry positive expression was CK19, EMA, and CEA, and for special staining periodic acid Shiff (PAS); 97% of cases were treated surgically. The average follow-up was 30.3 months, and recurrence was reported in 52.4% of the cases. Conclusion: CCOC shows a strong predilection for the body and anterior mandible, and females are more frequently affected. CCOCs can be painful and the principle clinical sign is swelling, CCOCs can metastasize, and the prognosis is fair.

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Clear cell odontogenic carcinoma is a rare, infrequent, aggressive in nature, locally reoccurring odontogenic tumor with a tendency of distant metastasis, occurring during to 4th to 6th decades with a mostly female predilection. Histologically, it is characterized by sheets and islands of vacuolated/clear cells. Till date, only 74 cases have been reported in the literature. We present a case of 45-year-old woman with a tumor mass extending from the maxillary right first premolar till the third molar region measuring 4 cm × 4 cm. The diagnosis was given based on the histopathological findings. Being locally aggressive, the reported data and understanding of this infrequent tumor needs to be strengthened by reporting new cases, and it also demands to be distinguished from other primary and metastatic clear cell tumors of the head-and-neck region.

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Clear Cell odontogenic Carcinomas (CCOC) are rare, aggressive malignant odontogenic tumours which are often misdiagnosed as benign odontogenic tumours due to the non-specific histologic appearance, and benign early clinical presentation. However, due to their propensity to metastasize, the best outcomes are experienced with they are diagnosed early and treated aggressively. In this paper, we present a case of a CCOC misdiagnosed as a clear cell calcifying epithelial odontogenic tumour which was only found to be a CCOC after cervical node metastasis. The original diagnosis was questioned and confirmed to be a CCOC by identification of the chromosomal translocation EWSR1 on fluorescence in situ hybridization. This has recently been described in CCOC and a wide variety of other mesenchymal and epithelial neoplasms. Previous reports have demonstrated EWSR1-ATF1 and EWSR1-CREB1 fusions in CCOC. Next generation sequencing of this case demonstrated the EWSR1-CREM fusion gene which has not been previously reported for CCOC. CREM fusion proteins have only recently been found in several tumour types including the closely associated hyalinizing clear cell carcinoma of salivary glands. This is discussed in this paper, and the role of the discovery of the CREM fusion protein in CCOC adds to your understating of the role of CREM in oncogenesis, and the possible link between CCOCs and hyalinizing clear cell carcinomas.

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Clear cell odontogenic carcinoma (CCOC) is a rare odontogenic tumor associated with aggressive clinical behavior, metastasis and low survival. To date, only 67 cases have been described in the English language literature, and an understanding of the behavior of CCOC has been based on limited case reports. The aim of the research was to further reveal the features of CCOC. We report 5 new cases of CCOC, with a mean age of 52.4 years. The clinical and histopathologic data of the disease obtained from earlier literature (95 cases) and the 5 new cases were analyzed. Data were extracted, including demographics, histopathologic findings, clinical presentation, primary treatment and outcomes. Immunohistochemical results revealed that the cancer is positive for AE1/AE3, EMA and CK19, negative for smooth muscle actin SMA, Vim and S-100. EWSR1 translocation was also observed in the new cases, which may help in the diagnosis of CCOC. Metastases of CCOC were rare, but the local recurrence rate of CCOC rose to 42%. The best treatment for patients with CCOC is wide local excision combined with regional lymph node dissection.

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Clear cell odontogenic carcinoma (CCOC) is a rare and aggressive malignant epithelial neoplasm, which occurs most frequently in the mandible of elderly patients. Morphologically, CCOC shares similar characteristics with other clear cell tumors, especially hyalinizing clear cell carcinoma of the salivary glands (HCCC). Both CCOC and HCCC are known to harbor EWSR1 rearrangements, especially the EWSR1-ATF1 gene fusion, which indicates a possible link between the two lesions. So far, this fusion has been demonstrated in five cases of CCOC in the literature. Herein, we add another CCOC case to the literature, which arose in the mandible of an 82-year-old female patient and was proven to harbor the EWSR1-ATF1 gene fusion. Immunohistochemically, this case was focally positive for CK7, CK14, CK19 and p63. The patient was referred to surgical treatment; however, she died of disease 2 months after the diagnosis, thereby demonstrating the aggressive nature of this tumor.

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The purpose of this study was to summarize the currently published cases of clear cell odontogenic carcinoma (CCOC). The PubMed and Springer databases were used to collect available reports, searching for 'clear cell odontogenic carcinoma', 'CCOC', or 'clear cell ameloblastoma'. The search resulted in 75 reports detailing 107 cases between 1985 and 2018. Clinically the tumor manifests as a swelling in the posterior mandible (n=46), anterior mandible (n=33), and maxilla (n=28). Radiological analysis of 85 cases typically showed a poorly defined expansive radiolucency (n=83). Of the 70 patients with symptoms reported, 44 specified a swelling, 11 tooth mobility, seven gingival/periodontal issues, five numbness, and three decreased jaw opening. One patient presented with a neck mass. The duration of symptoms prior to seeking care was specified for 52 patients: 2 months to 1 year for 34 patients, 1-2 years for seven, 2-4 years for two, 4-7 years for six, and 7-12 years for three. The incidence of recurrence appeared to be 38 of the 88 cases where recurrence was reported. CCOC can be distinguished from other oral cancers by its distinctive histology and immunohistochemical characteristics and less aggressive behavior. Currently, treatment should be early and aggressive resection with clear surgical margins and long-term follow-up. The overall goal is to collect a cohort of patients.

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Clear cells are associated with numerous physiological and pathological conditions. Odontogenic cysts and tumors can arise from physiological clear cells like rests of dental lamina. One such clear cell neoplasm is clear cell odontogenic carcinoma (CCOC) that is associated with aggressive clinical behavior, metastasis and low survival rate. With <80 cases reported so far, it is a rare entity. Initially described as a clear cell odontogenic tumor considering it as a benign but invasive neoplasm, it was later termed as CCOC owing to its infiltrative nature with a marked tendency for local recurrence, regional nodal spread and possible distant, mainly pulmonary, metastasis. Reported here is a case of CCOC affecting mandible of a 55-year-old female along with its immunohistochemical workup.

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Clear cell odontogenic carcinoma (CCOC), a rare tumor in the head and neck region, displays comparable properties with other tumors clinically and pathologically. In consequence, an incorrect diagnosis may be established. A 51-year-old male patient who was admitted to the Department of Oral and Maxillofacial Surgery at Pusan National University Dental Hospital was initially diagnosed with ameloblastoma via incisional biopsy. However, the excised mass of the patient was observed to manifest histopathological characteristics of ameloblastic carcinoma. The lesion was ultimately diagnosed as clear cell odontogenic carcinoma by the Department of Oral Pathology of Pusan National Dental University. Therefore, segmental mandibulectomy and bilateral neck dissection were performed, followed by reconstruction with fibula free flap and reconstruction plate. Concomitant chemotherapy radiotherapy was not necessary. The patient has been followed up, and no recurrence has occurred 6 months after surgery.

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Clear cell odontogenic carcinoma (CCOC) is a rare, low-grade malignant epithelial neoplasm, occurring in the jawbones, mainly affecting the mandible of elderly patients. In addition to hyalinizing clear cell carcinoma of the salivary gland, it is one of the epithelial neoplasms known to harbor an EWSR1-ATF1 fusion. Therefore, a link between these tumors seems plausible. We describe six cases of CCOC showing EWSR1 rearrangements, with two cases being positive for the ATF1 partner gene using FISH analysis. In one case, an EWSR1-CREB1 fusion was identified using RT-PCR, which we report for the first time in this tumor type. The other three cases investigated by FISH were negative for ATF1, CREB1 and CREB3L2. In conclusion, our data show that EWSR1-CREB1 is an alternative fusion gene to EWSR1-ATF1 in CCOC.

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Clear cell odontogenic carcinoma (CCOC), a rare tumor in the head and neck region, displays comparable properties with other tumors clinically and pathologically. In consequence, an incorrect diagnosis may be established. A 51-year-old male patient who was admitted to the Department of Oral and Maxillofacial Surgery at Pusan National University Dental Hospital was initially diagnosed with ameloblastoma via incisional biopsy. However, the excised mass of the patient was observed to manifest histopathological characteristics of ameloblastic carcinoma. The lesion was ultimately diagnosed as clear cell odontogenic carcinoma by the Department of Oral Pathology of Pusan National Dental University. Therefore, segmental mandibulectomy and bilateral neck dissection were performed, followed by reconstruction with fibula free flap and reconstruction plate. Concomitant chemotherapy radiotherapy was not necessary. The patient has been followed up, and no recurrence has occurred 6 months after surgery.

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