Rare clear cell odontogenic carcinoma associated with impacted tooth in a young patient: case report and literature review

Oral and Maxillofacial Surgery - Clear cell odontogenic carcinoma (CCOC) is a rare malignant odontogenic tumor. It is characterized by showing, on histopathological examination, clusters of...     

Atypical presentation of clear cell odontogenic carcinoma

ABSTRACT: Clear cell odontogenic carcinoma (CCOC) is a rare malignant neoplasm of odontogenic origin. The usual clinical presentation of CCOC is a mass of progressive growth in the mandible sometimes accompanied with loss of teeth, pain, or bleeding. We describe a rare case of CCOC that showed an atypical presentation not previously described in the literature like a fast-growing painless mass in the retromolar area that reached a considerable size in a few days that caused obstruction of the airway. The presence of airway obstruction required immediate treatment, which consisted of a surgical excision of the tumor via a hemimandibulectomy. This clinical report highlights the possibility of odontogenic tumors presenting like a rapid-growing mass and the importance of clinical differential diagnosis of such presentation.     

Clear Cell Odontogenic Carcinoma—A Rare Case Report

Clear cell odontogenic carcinoma (CCOC) is a rare odontogenic tumour with female predilection occurring in the anterior region of the mandible with peak age incidence of 5th and 7th decade of life. Here we report a case occurred in the posterior mandible of a 42 year old male patient which highlights the clinicopathologic features of CCOC that were confirmed by histopathologic examination. We add up yet another case of CCOC to the published literature.     

Clear cell odontogenic carcinoma and clear cell ameloblastoma: a single clinicopathologic entity? A new case and comparative analysis of the literature.

BACKGROUND: Clear cell odontogenic carcinoma (CCOC) is histologically characterized by solid sheets and nests of clear cells. Clear cell ameloblastoma (CCAM) is histologically characterized by an ameloblastomatous component intermixed with an extensive clear cell component. In all literature reviews, no separation has been made between the clinicopathologic features of CCOC and CCAM. PURPOSE: We sought to review and analyze the clinicopathologic and radiologic features and the biologic behavior of CCOC and to compare them with those of CCAM to evaluate the possible separation between the 2 lesions. MATERIALS AND METHODS: A MEDLINE search of the English-language literature was carried out for CCOC and CCAM. Cases were classified according to their histologic features. RESULTS: A total of 27 cases of CCOC (26 from the literature and 1 new case) and 8 cases of CCAM were found. CCOC patients showed a male-to-female ratio of 1:2.4, with a mean age of 59 years. CCAM patients showed a male-to-female ratio of 1.7:1, with a mean age of 44 years. CCOC and CCAM were predominantly found in the mandible. Both CCOC and CCAM showed a high rate of recurrence (50% and 63%, respectively) and metastases (33% and 25%, respectively). Several patients with CCOC presented with metastases at time of diagnosis, whereas patients with CCAM usually developed metastases only after several recurrences. CONCLUSION: Based on the relatively small number of cases in the literature on CCOC and CCAM, it is difficult to confidently separate the 2 lesions. Both lesions should be considered low-grade malignancies and could well represent a clinicopathologic continuum of a single disease entity rather than 2 separate lesions.     

Correlation between ploidy status using flow cytometry and nucleolar organizer regions in benign and malignant epithelial odontogenic tumors

ObjectiveDifferentiation between the aggressive benign odontogenic tumors and their malignant counterparts is controversial and difficult. While flow cytometry (FCM) allowed DNA analysis in neoplasia, argyrophilic organizer regions (AgNORs) number and/or size in a nucleus are correlated with the ribosomal gene activity and therefore with cellular proliferation. The aim of this research was to study the diagnostic accuracy of FCM and AgNORs staining in differentiating between benign and malignant epithelial odontogenic tumors and to correlate between these two interventions.DesignSixteen benign cases [8 cases of ameloblastoma (AB) and 8 cases of keratocystic odontogenic tumor (KCOT)] and 13 malignant epithelial odontogenic tumors [8 cases of ameloblastic carcinoma (ABC) and 5 cases of clear cell odontogenic carcinoma(CCOC)] were included in the current study. For FCM analysis, a single cell suspension from Formalin fixed paraffin-embedded (FFPE) tumors was prepared according to a modified method described by Hedley (1989) and AgNORs staining were performed in accordance to the Ploton protocol (1986). Analysis of AgNORs was performed using both quantitative and qualitative methods.ResultsThe work revealed that all the examined tumors were diploid, except for 40% of CCOC cases. The S-phase fraction (SPF) value, AgNORs count and AgNORs area/cell showed statistically significant difference on comparing benign and malignant groups. A weak positive correlation was observed between SPF and AgNORs count.ConclusionThe SPF value was considered to be more sensitive and specific in differentiation between aggressive benign and malignant epithelial odontogenic tumors in comparison to AgNORs counting.     

FDG-PET/CT in staging of clear cell odontogenic carcinoma

Clear cell odontogenic carcinoma (CCOC) is a rare neoplasm; only 75 cases have been reported in the English language literature. They have a tendency for recurrence and a capacity to metastasize. There is very little known regarding the metabolic features of this tumour or the utility of fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) scans in the staging and follow-up of these tumours. We present two cases of CCOC with their relevant FDG-PET/CT scan findings. The first patient had primary CCOC of the mandible that was FDG-avid, and the other had recurrence of CCOC of the anterior mandible and superomedial orbit that was not FDG-avid. FDG uptake in CCOC appears to be variable. Although FDG-PET/CT is useful in other head and neck cancers and has benefits compared to other imaging modalities, further studies are needed to investigate the sensitivity of FDG-PET/CT in CCOC.     

The central (intraosseous) calcifying odontogenic cyst: an analysis of 215 cases

This study reviews and analyzes the clinical, radiographic, and histomorphologic features of the 215 cases of central calcifying odontogenic cyst (CCOC) reported in the literature. Based on the present information, a clinicopathologic classification of calcifying odontogenic cyst is proposed. The CCOC is also compared with its peripheral counterpart and the differences discussed.     

Clear cell odontogenic carcinoma of the mandible.

This article describes a case of clear cell odontogenic carcinoma (CCOC) in a 55-year-old woman who presented such a tumor extending from the midline of the mandible to the right first molar. The tumor was surgically excised and has not recurred or metastasized 6 months after surgery. To date, only 33 well-documented cases have been reported (including the present case) in the English literature. Twenty-eight (84.8%) have developed in the mandible and five (15.2%) in the maxilla. Eighteen (54.5%) occurred anterior to the first molar. The vast majority have been diagnosed in patients older than 40 years (30/33), and the mean age at diagnosis is 57.4 years (range: 17-89), with a male/female ratio: 1/2. Recurrences have been described in 17 cases (51.5%) and metastasis in 10 (30.3%). Based on its morphologic, histochemical and immunophenotypic features, CCOC should be distinguished from other primary and metastatic clear cell tumors of the oral and maxillofacial regions.     

Clear cell odontogenic carcinoma: a rare jaw tumor. A summary of 107 reported cases

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.     

Clear cell odontogenic carcinoma in the mandible: histochemical and immunohistochemical observations with a review of the literature

A rare case of clear cell odontogenic carcinoma was investigated using histochemical and immunohistocheinical methods. The tumor occurred in the anterior mandible of a 69-year-old Japanese man. Histologically, the tumor was composed mostly of large clear cells and squamous cells. Columnar-shaped cells with basophilic nuclei polarized away from the basement membrane were observed at the periphery of the tumor foci. The tumor cells had aggressively invaded muscle and perineural tissues. The tumor cells were positive for PAS staining. Immunohistochemically, tumor cells reacted positively to keratin. Cytokeratin19, epithelial membrane antigen, and S-100 protein. The tumor was diagnosed as a clear cell odontogenic carcinoma. Its characteristics are discussed in term of its histopathological, histochemical and immunohistochemical features.     

量子点荧光探针检测人舌鳞状细胞癌荷瘤裸鼠模型早期下颌下淋巴结转移的研究

目的 利用量子点标记的特异性细胞角蛋白抗体QDs605-CK（AE1/AE3）免疫荧光探针检测人舌鳞状细胞癌荷瘤裸鼠早期下颌下淋巴结转移率及微转移率,并与传统的免疫组织化学（IHC）染色及苏木精-伊红（HE）染色方法进行比较,为舌鳞状细胞癌的早期诊断与治疗提供一种新的检测方法。方法 传代培养人舌鳞状细胞癌Tca8113 细胞,接种于18只裸鼠舌体内（不过中线）,建立人舌癌荷瘤裸鼠下颌下淋巴结转移模型。接种6周后,处死裸鼠,解剖下颌下淋巴结,将同一淋巴结分为两份。一份作石蜡包埋半连续切片,行HE染色和IHC检测;另一份即刻液氮冷冻,制作冰冻切片行QDs605-CK（AE1/AE3）荧光探针检测。分别计算3种方法检测出的淋巴结转移率和微转移率。结果 量子点标记的免疫荧光染色检测出裸鼠下颌下淋巴结转移率为66.7%,其中微转移率为38.9%;IHC染色检测的淋巴结转移率为61.1%,其中微转移率为33.3%;HE染色检测的淋巴结转移率为27.8%。经统计学分析,3种方法的差异有统计学意义（χ2=6.379,P<0.05）,量子点标记的免疫荧光染色和IHC检测都优于HE染色,但是量子点标记的免疫荧光染色和IHC染色间的差异无统计学意义（χ2=0.120,P>0.05）。结论 量子点标记的QDs605-CK（AE1/AE3）免疫荧光探针能准确定位于下颌下淋巴结转移的肿瘤细胞的细胞质内,发出红色荧光,其特异性强,分辨率高,背景清晰,能够用于淋巴结转移灶及微转移灶的检测。     

Immunohistochemical investigation in odontogenic myxoma

Three odontogenic myxomas are described immunohistochemically by a panel of poly- and monoclonal antibodies to characterize this tumor type. Three types of odontogenic myxoma cells were discriminated: spindle cells, stellate cells and hyaline cells. Neoplastic cells of myxomas were positively stained for transferrin, ferritin, alpha-1-antichymotrypsin (alpha 1-ACT), alpha-1-antitrypsin (alpha 1-AT), S-100 protein and vimentin; however, neuron specific enolase (NSE), S-100 alpha subunit, S-100 beta subunit, Factor VIII-related antigen (FVIII-AG) and cytokeratin (CK1) were negative. Spindle cells were positive for transferrin, ferritin, alpha 1-ACT, alpha 1-AT, S-100 protein and vimentin. Stellate cells were strongly positive for transferrin, alpha 1-AT, S-100 protein and vimentin. Hyaline cells reacted with alpha 1-ACT and alpha 1-AT. Myxomatous matrix showed negative reaction for all the antibodies used. These results have confirmed that odontogenic myxoma is a tumor of a dual fibroblastic-histiocytic origin.     

Central (intraosseous) calcifying odontogenic cyst

The central calcifying odontogenic cyst (CCOC) is an uncommon lesion. The present study reports 17 new cases of CCOC and analyses their clinical and histomorphologic features. The data revealed that CCOC were usually diagnosed in the second decade of life with an almost equal distribution between the maxilla and the mandible. Six of the 17 cases (35%) were associated with odontomas and 6 lesions (35%) were associated with unerupted teeth. Histologically, most of the lesions were unilocular cysts but at least one was multilocular and one was a mixed lesion - partially cystic and partially solid. Excision with long-term follow-up (up to 10 years) is the treatment of choice.     

A clinicopathologic study on basaloid squamous cell carcinoma in the oral and maxillofacial region

Basaloid squamous cell carcinoma (BSCC) is a rare distinct variant of squamous cell carcinoma (SCC). To investigate its clinical behavior and prognosis, 15 patients with BSCC in the oral and maxillofacial region were clinically analyzed and compared with 15 patients with conventional SCC matched for site, stage, gender and age. To understand its immunohistochemical features, sections for cytokeratin AE1/AE3, CK 13. CK 7, CK 8, proliferating cell nuclear antigen (PCNA) and p53 were reviewed from 12 patients with BSCC. The rate of cervical lymph node metastasis of BSCC was as high as 67% and that of distant metastasis 13%. The tumor recurrence rate was 33% and the 3-year and 5-year survival rates were 53% and 32%, respectively. For conventional SCC, the cervical lymph node metastasis rate was 27%, that of distant metastasis 7%, tumor recurrence rate was 33%, and 3-year and 5-year survival rates were 80% and 70%, respectively. In most BSCC patients (10/12) the PCNA index was over 50%. Twelve BSCC patients were diagnosed with grade II or III conventional SCC when the original records of the primary diagnosis for the 15 patients with BSCC were reviewed. The biological behavior and prognosis of BSCC are similar to those of poorly differentiated SCC.     

Peripheral ameloblastoma with potentially malignant features: report of a case with special regard to its keratin profile

A peripheral ameloblastoma with atypical features occurring on the left maxillary alveolar ridge of 40-year-old man is described, along with an immunohistochemical profile of its cytokeratin (CK). The lesion apparently originated from the surface gingival epithelium. The tumor nests or strands were highly cellular with a variable degree of squamous differentiation and microcyst formation. Occasional mitotic figures and dystrophic calcification, both of which are not seen in conventional ameloblastomas, were also observed. The tumor infiltrated deep into the alveolar mucosa, including the periodontal ligament, and showed histological and topographical evidence of atypism, resulting in resorption of the underlying alveolar bone. On the CK immunohistochemistry, CK19 was demonstrated in all the types of neoplastic epithelia, including microcyst-forming cells, densely packed round or spindle cells within the tumor nests, cells with squamous metaplasia, and peripheral tall columnar cells. The CK immunohistochemical findings suggest the lesion's cell of odontogenic origin; they may reflect an immature phenotypic expression of cell differentiation in the odontogenic epithelia during the tumor growth in the gingival mucosa.     

牙源性上皮性联合瘤的临床病理和角蛋白表达研究

目的：研究4例牙源性上皮性联合瘤的临床病理和角蛋白免疫组化表达。方法：4例牙源性上皮性联合瘤均来源于武汉大学口腔医学院病理学教研室，10％福尔马林溶液固定，常规制片HE染色，免疫组化SP法检测肿瘤组织中CKl0、CKl0和13、CKl8和8、CKl9蛋白的表达。结果：全部4例中均显示牙源性腺样瘤的典型组织学特点，牙源性钙化上皮瘤样区大小不等，1例中的CEOT区为主要成分，CKl9呈阳性表达。结论：报告四例牙源性腺样瘤和牙源性钙化上皮瘤构成的牙源性上皮性联合瘤，其中一例为罕见的以牙源性钙化上皮瘤成分为主。     

Peripheral clear cell variant of calcifying epithelial odontogenic tumor: Report of a case and immunohistochemical investigation

A case of peripheral calcifying epithelial odontogenic tumor, clear cell variant, located on the right gingival maxilla of a 48-year-old woman, presenting as a 2.0-cm solitary, firm nodule was studied. Microscopically, it was composed of polyhedral and clear epithelial cells associated with amyloid-like deposition. The clear epithelial cells exhibited granules that were positive for periodic acid-Schiff, and the amyloid-like deposit stained with Congo red showed a green birefringence in the polarized light. Polyhedral and clear epithelial cells were immunopositive for AE1/AE3 and cytokeratin 14. Immunoexpression of fibronectin and types I and III collagen were different between the amyloid-like deposits and the connective tissue stroma. Tenascin surrounded epithelial cells located inside the amyloid-like deposits. Laminin and type IV collagen were immunodetectable around the strands, cords, and nests of epithelial cells. This report represents the seventh case of peripheral calcifying epithelial odontogenic tumor, clear cell variant.     

Clinicopathological and immunohistochemical features of oral spindle cell carcinoma

J Oral Pathol Med (2010) 39 : 335–341 Background: Oral spindle cell carcinoma (SpCC) is a rare variant of oral squamous cell carcinoma (SCC). The aims of this study were to compare the clinicopathologic and immunohistochemical features of oral SpCC with conventional oral SCC. Methods: Five cases of oral SpCC and 10 cases of oral SCC (five well‐differentiated and five poorly differentiated) were evaluated through conventional hematoxylin and eosin staining and immunohistochemical reactions to cytokeratins (CK), vimentin, desmin, smooth muscle actin, muscle‐specific actin, S‐100 protein, epithelial membrane antigen (EMA), p53, and ki‐67. Results: Oral SpCC showed predilection for males on their sixth decade of life, presenting clinically as painful infiltrative ulcers or ulcerated exophytic polypoid masses, preferably located on the alveolar mucosa. Mesenchymal markers were expressed in the spindle cell but not in the carcinomatous component of SpCC, and it was negative in all SCC. CKs AE1/AE3, 6, 14, and EMA were positive on both carcinomatous and spindle cell components of most SpCCs. These tumors also presented higher p53 and ki‐67 expression and no CK 1 expression in contrast to well‐differentiated SCC. Conclusion: Oral SpCC presented a different clinical profile than conventional SCC and histopathologic features and p53 and ki‐67 expression closer to poorly differentiated SCC. Besides mesenchymal markers, CK AE1/AE3, 6, 14, and EMA expression on spindle cells may be useful as an adjunct on microscopical differential diagnosis of SpCC.     

Proliferating cell nuclear antigen (PCNA) and p53 protein expression in ameloblastoma and adenomatoid odontogenic tumor

In this study, proliferating cell nuclear antigen (PCNA) and p53 protein expressions were analyzed in 16 cases of ameloblastoma and 8 cases of adenomatoid odontogenic tumor (AOT). The cases of ameloblastoma consisted of solid type tumors and histologic arrangements of different subtypes were observed. In some specimens, more than one histologic subtype was identified in the same lesion, and each tumor was categorized according to the predominant cell pattern. The odontogenic tumors were grouped as follows: follicular ameloblastoma (n=7), plexiform ameloblastoma (n=4), acanthomatous + follicular ameloblastoma (n=3), basal cell ameloblastoma (n=2), adenomatoid odontogenic tumor (n=8). PCNA immunohistochemical expression revealed stronger quantitative labeling index for the follicular ameloblastoma, while for p53 protein the strongest quantitative labeling index was detected in the plexiform type. Nevertheless, statistical analysis using ANOVA and Tukey's test did not detect significant differences (p>0.05) among the histologic subtypes of ameloblastoma. The findings of this study suggest that the different histologic patterns of ameloblastoma did not show a direct correlation with their clinical behavior and consequently with the prognosis of the cases. The results also indicated that the ameloblastoma has greater proliferative potential than the AOT, which can contribute to explain its more aggressive and invasive characteristics.