Sclerosing odontogenic carcinoma misnomering previously as central odontogenic fibroma-A case report with review of literature.

Sclerosing odontogenic carcinoma (SOC) was first described by Koutlas et al. in 2008. Despite its inclusion in the World Health Organization (WHO) as a distinct entity, it is a tumour that remains poorly defined in the literature, with only 10 reported cases to date. The mandibular premolar and molar region is more commonly affected compared to the maxilla. In the maxilla, the anterior and the molar regions are most commonly affected. This article describes a case report of a Sclerosing Odontogenic Carcinoma in a 50 year old male patient in the mandibular region. The radiograph showed a well-defined radiolucency extending from the left ramus of the mandible to the right lower molar region. SOC is low grade with mild atypia and frequent mitosis and diffused infiltrative and perineural spread.

Sclerosing Odontogenic Carcinoma With a Prominent Clear Cell Component Mimicking Odontogenic Clear Cell Carcinoma: An Extremely Rare Case With a Fatal Clinical Outcome.

Sclerosing odontogenic carcinoma (SOC) is an exceedingly rare odontogenic carcinoma known for its locally aggressive yet indolent behavior. There have been no reports of metastasis to distant organs, except a single case involving lymph node metastasis. This report details the case of a 49-year-old female who presented with a well-demarcated radiolucent lesion in the mandible, accompanied by root resorption and tooth displacement. Microscopically, the lesion exhibited a distinctive composition, with two distinct components: cords of epithelium embedded within an abundant collagenous stroma and solid nests of clear polygonal cells surrounded by hyalinized stroma. Notably, the tumor exhibited direct invasion into the submental muscles, accompanied by perineural and vascular invasion, as well as cortical bone loss. Additionally, the clear cells contained diastase-sensitive periodic acid-Schiff-positive granules. Immunohistochemically, the tumor cells displayed positivity for cytokeratin 19 and p63 while testing negative for myoepithelial markers. The Ki-67 index was measured at 23%. Importantly, neitherEWSR1 nor MAML2 rearrangements were detected through fluorescence in situ hybridization (FISH) analysis. Over several years, this patient experienced three instances of local recurrence; notably, four years after the initial surgery, fludeoxyglucose F18-positron emission tomography (18FDG-PET)/CT scans confirmed the presence of pulmonary metastasis. This case presents an unusual histological variation of SOC, marked by vascular invasion, and is notably the first documented case of a fatal outcome in this context.

A case of sclerosing odontogenic carcinoma of the mandible with a review of the literature.

Sclerosing odontogenic carcinoma (SOC) is a very rare malignant odontogenic tumor characterized by sclerotic stroma and single-file cord-like tumor cell structures. A 38-year-old man presented with extraoral swelling and right mental region paralysis. Panoramic radiography revealed an ill-defined radiolucent lesion extending from the right mandibular ramus to the right lower canine. Magnetic resonance imaging showed tumor invasion into the right inferior alveolar nerve and masseter muscle. Hemimandibulectomy, bilateral neck dissection, and mandibular reconstruction were performed using a rectus abdominis musculocutaneous flap and a titanium plate. Histopathology and immunohistochemistry confirmed SOC diagnosis. No recurrence occurred in the 1-year follow-up. In this paper, a case of SOC with a high Ki-67 labeling index was reported. Since SOC is prone to nerve invasion, treatment is resection with an appropriate surgical margin.

Proceedings of the 2023 North American Society of Head and Neck Pathology Companion Meeting, New Orleans, LA, March 12, 2023: Odontogenic Tumors: Have We Achieved an Evidence-Based Classification.

BACKGROUND: The World Health Organization's (WHO) chapter on odontogenic and maxillofacial bone tumors provides a global reference for diagnosis of these tumors. In the fifth edition, the inclusion of consensus definitions and development of essential and desirable diagnostic criteria help improve recognition of distinct entities. These are key enhancements since the diagnosis of odontogenic tumors is largely based on histomorphology which is taken in combination with clinical and radiographic appearances. METHODS: Review. RESULTS: Despite delineation of diagnostic criteria for ameloblastoma, adenoid ameloblastoma, and dentinogenic ghost cell tumor, a subset of these tumors continues to show overlapping histological features that can potentially lead to misdiagnosis. Accurate classification may be challenging on small biopsies, but potentially enhanced by refining existing diagnostic criteria and utilization of immunohistochemistry and/or molecular techniques in a specific cases. It has become clear that the clinical and histologic features of the non-calcifying Langerhans cell-rich subtype of calcifying epithelial odontogenic tumor and the amyloid-rich variant of odontogenic fibroma converge into a single tumor description. In addition, this tumor shows remarkable clinical, histological overlap with a subset of sclerosing odontogenic carcinoma located in the maxilla. Benign perineural involvement vs perineural invasion is an underexplored concept in odontogenic neoplasia and warrants clarification to reduce diagnostic confusion with sclerosing odontogenic carcinoma. CONCLUSION: While controversial issues surrounding classification and discrete tumor entities are addressed in the WHO chapter, ambiguities inevitably remain. This review will examine several groups of odontogenic tumors to highlight persistent knowledge gaps, unmet needs and unresolved controversies.

Sclerosing odontogenic carcinoma of maxilla: A case report.

BACKGROUND: Sclerosing odontogenic carcinoma is a rare primary intraosseous neoplasm that was featured recently as a single entity in the World Health Organization classification of Head and Neck Tumors 2017, with only 14 cases published to date. The biological characteristics of sclerosing odontogenic carcinoma remain indistinct because of its rarity; however, it appears to be locally aggressive, with no regional or distant metastasis reported to date. CASE SUMMARY: We reported a case of sclerosing odontogenic carcinoma of the maxilla in a 62-year-old woman, who presented with an indolent right palatal swelling, which progressively increased in size over 7 years. Right subtotal maxillectomy with surgical margins of approximately 1.5 cm was performed. The patient remained disease free for 4 years following the ablation surgery. Diagnostic workups, treatment, and therapeutic outcomes were discussed. CONCLUSION: More cases are needed to further characterize this entity, understand its biological behavior, and justify the treatment protocols. Resection with wide margins of approximately 1.0 to 1.5 cm is proposed, while neck dissection, post-operative radiotherapy, or chemotherapy are deemed unnecessary.

Sclerosing Odontogenic Carcinoma: Report of a Case and Review of the Literature.

BACKGROUND: Sclerosing odontogenic carcinoma is an exceedingly rare gnathic malignancy first described by Koutlas et al. in 2008, and was only recently designated as a distinct pathologic entity by World Health Organization in the 2017 Classification of Head and Neck Tumors. To date, fewer than fifteen cases of this neoplasm have been reported in the English language literature. This tumor is characterized by thin cords, strands, and small nests of epithelium in a densely sclerotic stroma. In some tumor foci, the density of the stroma may be sufficient to compress the epithelial component beyond detection in the absence of immunohistochemistry, thus rendering this entity a particularly challenging diagnosis in small sample sizes. METHODS: A 55-year-old male presented with an asymptomatic lesion of posterior left maxilla. Cone beam computed tomography (CBCT) demonstrated a large, well-defined bony lesion with scalloped border, spanning from canine to first molar. External root resorption of the adjacent teeth was also noted. Microscopic examination of the biopsy specimen revealed an odontogenic tumor with features consistent with sclerosing odontogenic carcinoma. Immunohistochemical staining was performed to confirm the diagnosis. RESULTS: The tumor was positive for CK5/6, CK19, E-cadherin, p63 and negative for CK20 and CK7. CONCLUSION: Sclerosing odontogenic carcinoma is a rare, low-grade malignancy of odontogenic origin, which represents a diagnosis of exclusion in many cases. An immunohistochemical profile demonstrating positivity for markers including CK5/6, CK19, p63, and E-cadherin, in addition to a set of pertinent negative findings, can aid in the diagnosis of this tumor. This entity appears to lack metastatic potential despite its locally destructive behavior and a common histologic finding of perineural invasion.

Maxillary clear cell odontogenic carcinoma with EWSR1-ATF1 fusion gene mimicking sclerosing odontogenic carcinoma: A case report and literature review.

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.

Sclerosing odontogenic carcinoma — review of all published cases: is it a justifiable addition as a malignancy? / Carcinoma odontogênico esclerosante - revisão de todos os casos publicados: sua classificação como tumor maligno é justificável?

Abstract Introduction Sclerosing odontogenic carcinoma was a new addition to the list of head and neck tumors by World Health Organization in 2017. This lesion has scarcely been reported and a lack of pathognomonic markers for diagnosis exists. Objective The aim of the study was to summarize findings from the available literature to provide up-to-date information on sclerosing odontogenic carcinoma and to analyse clinical, radiological, and histopathological features to obtain information for and against as an odontogenic malignancy. Methods We conducted a comprehensive review of literature by searching Pubmed, EBSCO and Web of Science databases, according to PRISMA guidelines. All the cases reported as sclerosing odontogenic carcinoma in English were included. Data retrieved from the articles were gender, age, clinical features, site, relevant medical history, radiographical findings, histopathological findings, immunohistochemical findings, treatments provided and prognosis. Results Mean age at diagnosis of sclerosing odontogenic carcinoma was 54.4 years with a very slight female predilection. Sclerosing odontogenic carcinoma was commonly reported in the mandible as an expansile swelling which can be asymptomatic or associated with pain or paraesthesia. They appeared radiolucent with cortical resorption in radiograph evaluation. Histologically, sclerosing odontogenic carcinoma was composed of epithelioid cells in dense, fibrous, or sclerotic stroma with equivocal perineural invasion. Mild cellular atypia and inconspicuous mitotic activity were observed. There is no specific immunohistochemical marker for sclerosing odontogenic carcinoma. AE1/AE3, CK 5/6, CK 14, CK19, p63 and E-cadherin were the widely expressed markers for sclerosing odontogenic carcinoma. Surgical resection was the main treatment provided with no recurrence in most cases. No cases of metastasis were reported. Conclusion From the literature available, sclerosing odontogenic carcinoma is justifiable as a malignant tumor with no or unknown metastatic potential which can be adequately treated with surgical resection. However, there is insufficient evidence for histological grading or degree of malignancy of this tumor.

Resumo Introdução O carcinoma odontogênico esclerosante é a nova adição à lista de tumores de cabeça e pescoço da Organização Mundial da Saúde em 2017. Essa lesão é pouco relatada e não há marcadores patognomônicos para o diagnóstico. Objetivo Resumir os achados da literatura disponível para fornecer informações atualizadas sobre o carcinoma odontogênico esclerosante e analisar as características clínicas, radiológicas e histopatológicas a favor e contra sua classificação como uma lesão odontogênica maligna. Método Uma revisão abrangente da literatura foi feita nos bancos de dados Pubmed, Ebsco e Web of Science, de acordo com as diretrizes do Prisma. Todos os casos relatados em inglês como carcinoma odontogênico esclerosante foram incluídos. Os dados recuperados dos artigos foram sexo, idade, características clínicas, sítio do tumor, histórico médico relevante, achados radiográficos, achados histopatológicos, achados imuno-histoquímico, tratamentos instituídos e prognóstico. Resultados A média de idade ao diagnóstico de carcinoma odontogênico esclerosante foi de 54,4 anos, com uma predileção muito leve pelo sexo feminino. Tumores do tipo carcinoma odontogênico esclerosante foram comumente relatados na mandíbula como um edema expansivo, que pode ser assintomático ou associado a dor ou parestesia. Eles têm aparência radiolucente com reabsorção cortical na radiografia. Histologicamente, o carcinoma odontogênico esclerosante é composto por células epitelioides em estroma denso, fibroso ou esclerótico com invasão perineural ambígua. Atipia celular leve e atividade mitótica imperceptível foram observadas. Não há um marcador imuno-histoquímico específico para SOC. AE1/AE3, CK 5/6, CK 14, CK19, p63 e E-caderina foram os marcadores amplamente expressos para carcinoma odontogênico esclerosante. A ressecção foi o principal tratamento feito sem recorrência na maioria dos casos. Nenhum caso de metástase foi relatado. Conclusão De acordo com a literatura disponível, é justificável classificar o carcinoma odontogênico esclerosante como um tumor maligno com nenhum ou desconhecido potencial metastático, que pode ser tratado adequadamente com ressecção cirúrgica. Entretanto, não há evidências suficientes para a graduação histológica ou de malignidade desse tumor.

Sclerosing odontogenic carcinoma - review of all published cases: is it a justifiable addition as a malignancy?

INTRODUCTION: Sclerosing odontogenic carcinoma was a new addition to the list of head and neck tumors by World Health Organization in 2017. This lesion has scarcely been reported and a lack of pathognomonic markers for diagnosis exists. OBJECTIVE: The aim of the study was to summarize findings from the available literature to provide up-to-date information on sclerosing odontogenic carcinoma and to analyse clinical, radiological, and histopathological features to obtain information for and against as an odontogenic malignancy. METHODS: We conducted a comprehensive review of literature by searching Pubmed, EBSCO and Web of Science databases, according to PRISMA guidelines. All the cases reported as sclerosing odontogenic carcinoma in English were included. Data retrieved from the articles were gender, age, clinical features, site, relevant medical history, radiographical findings, histopathological findings, immunohistochemical findings, treatments provided and prognosis. RESULTS: Mean age at diagnosis of sclerosing odontogenic carcinoma was 54.4 years with a very slight female predilection. Sclerosing odontogenic carcinoma was commonly reported in the mandible as an expansile swelling which can be asymptomatic or associated with pain or paraesthesia. They appeared radiolucent with cortical resorption in radiograph evaluation. Histologically, sclerosing odontogenic carcinoma was composed of epithelioid cells in dense, fibrous, or sclerotic stroma with equivocal perineural invasion. Mild cellular atypia and inconspicuous mitotic activity were observed. There is no specific immunohistochemical marker for sclerosing odontogenic carcinoma. AE1/AE3, CK 5/6, CK 14, CK19, p63 and E-cadherin were the widely expressed markers for sclerosing odontogenic carcinoma. Surgical resection was the main treatment provided with no recurrence in most cases. No cases of metastasis were reported. CONCLUSION: From the literature available, sclerosing odontogenic carcinoma is justifiable as a malignant tumor with no or unknown metastatic potential which can be adequately treated with surgical resection. However, there is insufficient evidence for histological grading or degree of malignancy of this tumor.

An extensive sclerosing odontogenic carcinoma in mandible: a case report and literature review.

Sclerosing odontogenic carcinoma (SOC) is a primary intraosseous carcinoma of the jaw that was listed as a separate entity for the first time in the latest version of the World Health Organization classification of Head and Neck Tumors (2017). In this report, we present a case of SOC involving a circuitous diagnostic process because of the inadequately detailed biopsy findings and inherent impression based on the imaging manifestations. Through an extensive literature review, the histopathological and immunohistochemical features of the disease were briefly summarized. Radiological findings of SOC have been characterized in detail, and an imaging classification scheme has been proposed to further discuss the diversity of radiographic features. Due to the rarity of the disease, a comprehensive understanding of SOC is needed, and close collaboration between clinicians, radiologists, and pathologists is crucial to avoid misdiagnosis.

Sclerosing Odontogenic Carcinoma with Local Recurrence: Case Report and Review of Literature.

Sclerosing odontogenic carcinoma is a rare locally destructive neoplasm with many histologic mimics. Here the diagnostic challenges are presented of a case of sclerosing odontogenic carcinoma with variable histologic features, including unusual and unexpected negative immunostaining for CK19.

Sclerosing odontogenic carcinoma: current diagnostic and management considerations concerning a most unusual neoplasm.

Sclerosing odontogenic carcinoma (SOC) is a primary intraosseous carcinoma of the jaws that has been listed as a separate entity for the first time in the latest version of the World Health Organization classification of Head and Neck Tumours (2017). The aim of this study was to analyse and interpret the existing literature on SOC in the context of a clinical case treated in the authors' department. A systematic search of the PubMed database was performed in accordance with the PRISMA guidelines, yielding nine cases of SOC reported so far. In summary, characteristic clinical and radiological features of SOC include asymptomatic swelling, location predominantly in the mandible, tumour primarily lytic in appearance, presence of cortical bone destruction, and lack of metastatic spread. Due to the rarity of the disease, close collaboration between oral/maxillofacial surgeons and pathologists is crucial to avoid misdiagnosis. With complete excision, no recurrence of SOC should be expected.