Mandible & maxilla
Malignant odontogenic tumors
Sclerosing odontogenic carcinoma
Authors: Nathan Lee, D.M.D., Tony Ng, M.D., Ph.D.
Editor-in-Chief: Debra L. Zynger, M.D.
Last author update: 13 April 2020
Last staff update: 24 August 2022
Copyright: 2020-2024, PathologyOutlines.com, Inc.
PubMed search: sclerosing odontogenic carcinoma
Table of Contents
Definition / general | Essential features | ICD coding | Epidemiology | Sites | Pathophysiology | Etiology | Clinical features | Diagnosis | Radiology description | Radiology images | Prognostic factors | Case reports | Treatment | Clinical images | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Sample pathology report | Differential diagnosis | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Lee N, Ng T. Sclerosing odontogenic carcinoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/mandiblemaxillasclerosingodontogenic.html. Accessed April 19th, 2024.
Definition / general
- Relatively new entity recently defined in the 2017 WHO classification of head and neck tumors, 4th edition (El-Naggar: WHO Classification of Head and Neck Tumours, 4th Edition, 2017)
- Very rare, fewer than 15 case reports have been described in the literature as of January 2020
- Currently regarded as diagnosis of exclusion due to clinical and histologic similarity to other head and neck entities (Head Neck Pathol 2019;13:371)
- Locally aggressive malignancy with low metastatic potential (Am J Surg Pathol 2008;32:1613, Oral Surg 2019;12:133)
Essential features
- Rare, newly defined odontogenic malignancy that is a diagnosis of exclusion
- Variable radiographic appearance but has the invasive and destructive features of a malignancy
- Markedly sclerotic stroma containing bland epithelial cords to nests but with infiltrative margins
ICD coding
Epidemiology
- M = F
- Affects adults with wide age range from 30 to > 70 years old (Am J Surg Pathol 2008;32:1613, Am J Surg Pathol 2008;32:1613)
Sites
- Mandible > maxilla (Int J Oral Maxillofac Surg 2017;46:1641)
- Typically premolar and molar regions in the mandible
- Typically anterior or molar regions in the maxilla
Pathophysiology
- Neoplastic but exact pathophysiology is not known at this time
Etiology
- Currently unclear
- Potential sources of origin include odontogenic epithelial rests (dental lamina Rests of Serres, Hertwig epithelial root sheath, rests of Malassez) and malignant transformation of odontogenic cyst epithelial lining (Am J Surg Pathol 2008;32:1613, Oral Surg Oral Med Oral Pathol Oral Radiol 2016;122:e204)
- Aggressive nature may be associated with a specific genetic etiology, which has not yet been identified (Int J Oral Maxillofac Surg 2017;46:1641, J Oral and Maxillofacial Surg Med Pathol 2018;30:428)
- Marked sclerosis in the surrounding stroma may play a role in the low rate of metastasis (Pathol Int 2010;60:694, J Oral and Maxillofacial Surg Med Pathol 2018;30:428)
Clinical features
- May have expansion of the surrounding jawbone and increased mobility of involved teeth (Am J Surg Pathol 2008;32:1613)
- May have paresthesia, especially if the tumor involves the inferior alveolar nerve or mental nerve (Oral Surg Oral Med Oral Pathol Oral Radiol 2013;116:e283, J Oral and Maxillofacial Surg Med Pathol 2018;30:428)
Diagnosis
- Based on a combination of clinical examination and definitive diagnosis requires correlation between both radiographic and histologic findings
Radiology description
- Poorly defined radiolucency but there may be a mixed radiolucent-radiopaque character that radiographically mimics benign fibro-osseous lesions such as fibrous dysplasia or cemento-osseous dysplasia (Int J Oral Maxillofac Surg 2017;46:1641, Head Neck Pathol 2019;13:371, Pathol Int 2010;60:694)
- Tumor has been reported to extend beyond radiographic margins (J Oral and Maxillofacial Surg Med Pathol 2019 Nov 25 [Epub ahead of print])
- Frequently involves cortical bone causing thinning and may trigger root resorption and periapical bone loss of adjacent teeth (Oral Surg Oral Med Oral Pathol Oral Radiol 2013;116:e283)
- Generally displays localized but aggressive growth and destruction, possibly extending into the surrounding soft tissues (Oral Surg Oral Med Oral Pathol Oral Radiol 2016;122:e204)
Radiology images
Images hosted on other servers:
Infiltrative ground glass radiolucency
Prognostic factors
- Slow growing malignancy with good prognosis and rare metastasis (Am J Surg Pathol 2008;32:1613)
- Marginal status, specifically the peripherally infiltrative tumor cords and nests may be associated with recurrence (Am J Surg Pathol 2008;32:1613)
- Three cases of recurrence have been reported (Pathol Int 2010;60:694, Head Neck Pathol 2019;13:371, J Oral and Maxillofacial Surg Med Pathol 2019 Nov 25 [Epub ahead of print])
Case reports
- 31 year old woman with mass at previously extracted lower right first molar (Am J Surg Pathol 2008;32:1613)
- 43 year old woman with a soft tissue lesion at the anterior hard palate (Oral Surg Oral Med Oral Pathol Oral Radiol 2016;122:e204)
- 43 year old woman with radiolucency at the right mid anterior maxilla (Oral Surg 2019;12:133)
- 48 year old man with diffuse recurrent right mandibular expansion (J Oral and Maxillofacial Surg Med Pathol 2019 Nov 25 [Epub ahead of print])
- 54 year old man with radiolucency associated with vital upper right lateral incisor and canine (Oral Surg Oral Med Oral Pathol Oral Radiol 2013;116:e283)
- 60 year old man with radiolucency 4 weeks after extraction of a left mandibular third molar (Int J Oral Maxillofac Surg 2017;46:1641)
- 62 year old man with hepatocellular carcinoma and 6 month progressive left maxillary swelling (Head Neck Pathol 2019;13:371)
- 67 year old man with left lower lip and mental region paresthesia (Pathol Int 2010;60:694)
- 68 year old woman with mandibular gingival mass around a vital central incisor (J Oral and Maxillofacial Surg Med Pathol 2018;30:428)
- 73 year old woman with enlargement of the right maxilla by a radiolucent lesion (Am J Surg Pathol 2008;32:1613)
Treatment
- Due to limited literature, there is no consensus treatment (Head Neck Pathol 2019;13:371, Int J Oral Maxillofac Surg 2017;46:1641, Oral Surg 2019;12:133)
- Surgery appears to be the main curative modality but there is no agreement on the need for neck dissection given the low grade histologic appearance but high prevalence for perineural invasion (Oral Surg Oral Med Oral Pathol Oral Radiol 2013;116:e283)
- Adjuvant radiotherapy appears to be reserved for cases with positive surgical margins or recurrence; chemotherapy has also been used (Am J Surg Pathol 2008;32:1613, Head Neck Pathol 2019;13:371, Pathol Int 2010;60:694, J Oral and Maxillofacial Surg Med Pathol 2019 Nov 25 [Epub ahead of print])
Clinical images
Images hosted on other servers:
Anterior mandible exophytic mass
Gross description
- Firm expansile mass with poorly defined pushing borders (Head Neck Pathol 2019;13:371)
- Erosion of surrounding bone and teeth, if present (Am J Surg Pathol 2008;32:1613)
- May have infiltration into adjacent structures such as muscle, fat and sinus membranes (Oral Surg Oral Med Oral Pathol Oral Radiol 2016;122:e204)
- Definitive delineation between resection margin and tumor may be difficult to distinguish
Gross images
Images hosted on other servers:
Mandible tumor involving skin
Microscopic (histologic) description
- Bland epithelial cells of odontogenic origin without keratin differentiation in a background of sclerotic connective tissue stroma (Am J Surg Pathol 2008;32:1613, Oral Surg 2019;12:133)
- Cytoplasm may appear clear (Am J Surg Pathol 2008;32:1613, J Oral and Maxillofacial Surg Med Pathol 2019 Nov 25 [Epub ahead of print])
- Tumor cells range from cords of single file epithelial cells to nests mimicking odontogenic rests (Oral Surg Oral Med Oral Pathol Oral Radiol 2016;122:e204)
- The abundant eosinophilic stroma may be so predominant that it compresses the tumor cells, making them difficult to identify on hematoxylin and eosin staining alone (Am J Surg Pathol 2008;32:1613)
- Mitoses and nuclear pleomorphism are rare (Am J Surg Pathol 2008;32:1613, J Oral and Maxillofacial Surg Med Pathol 2018;30:428)
- Invasion of adjacent cortical bone, tooth roots, musculature, as well as perineural and perivascular invasion are common despite the low grade histology (Oral Surg Oral Med Oral Pathol Oral Radiol 2013;116:e283, Oral Surg 2019;12:133)
- Diagnosis is extremely difficult on small biopsies and the histology mimics other head and neck neoplasms (Int J Oral Maxillofac Surg 2017;46:1641, Oral Surg Oral Med Oral Pathol Oral Radiol 2013;116:e283)
- Overall cytologically bland but architecturally malignant and invasive
Microscopic (histologic) images
Contributed by Nathan Lee, D.M.D.
Architectural invasion
Bland cytology
Hypercellular tumor
Invasive clear cells
Hypercellular clear cell variant
Bland clear cells
Pankeratin
CK7
CK20
p63
Positive stains
- CK5/6
- p63
- E-cadherin
- CK19 (but two case reports were negative) (Head Neck Pathol 2019;13:371, J Oral and Maxillofacial Surg Med Pathol 2019 Nov 25 [Epub ahead of print])
Negative stains
- CK7 (may show focal positivity) (Head Neck Pathol 2019;13:371)
- CK20
- CK8 / CK18
- S100
- SMA
- Desmin
- CEA
- PAS-D / mucicarmine (Am J Surg Pathol 2008;32:1613, Pathol Int 2010;60:694)
Sample pathology report
- Left maxilla, resection:
- Sclerosing odontogenic carcinoma (see synoptic report)
Differential diagnosis
- Benign fibro-osseous lesion (fibrous dysplasia, cemento-osseous dysplasia):
- Radiologic margins should be neither poorly defined nor destructive
- Histology is predominantly osteoid / dentoid / cementoid material without sclerotic stroma or epithelial cells
- No recurrence of these lesions even on curettage
- Desmoplastic ameloblastoma:
- Tumor cells have reverse polarization of the nuclei along with peripheral palisading
- Stellate reticulum shows loose connective tissue
- Calcifying epithelial odontogenic tumor (CEOT):
- Has amyloid-like (odontogenic ameloblast associated protein) concentric deposits (Congo red apple green birefringence) and more scanty hyalinized stroma
- Squamous odontogenic tumor:
- Round to oval nests of squamous epithelium are much larger with occasional cystic degeneration
- Non-hyalinized stroma
- Central odontogenic fibroma, WHO type / epithelium rich variant (subtype removed in WHO Classification of Head and Neck Tumours, 4th Edition, 2017):
- Stroma has fibroblastic proliferation without sclerosis and has dystrophic calcification resembling dentin or cementum
- Central calcifying odontogenic cyst (CCOC):
- Tumor cells have vacuolated clear cytoplasm with eccentric dark nuclei with only a few eosinophilic cells
- EWSR1-ATF1 gene fusion
- Primary intraosseous odontogenic carcinoma:
- Tends to have much more cytologic atypia
- Metastatic disease:
- Most likely of epithelial origin, must be ruled out
Board review style question #1
A 62 year old male presented with a destructive and invasive odontogenic lesion involving the left maxilla and sinus. Which of the following immunohistochemical stain results would you expect from this sclerosing odontogenic carcinoma?
- CD117 positive
- CK20 positive
- Desmin positive
- p63 positive
- S100 positive
Board review style answer #1
Board review style question #2
A 62 year old male presented with a destructive and invasive lesion involving the midline maxilla and left sinus. Which of the following entities would not be on the differential diagnosis?
- Metastatic squamous cell carcinoma
- Olfactory neuroblastoma
- Primary intraosseous odontogenic carcinoma
- Sclerosing odontogenic carcinoma
- Squamous odontogenic carcinoma
Board review style answer #2
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