Table of Contents
Definition / general | Terminology | Epidemiology | Sites | Pathophysiology | Clinical features | Radiology description | Prognostic factors | Case reports | Treatment | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Electron microscopy description | Molecular / cytogenetics description | Differential diagnosisCite this page: Morrison, A. Calcifying odontogenic cyst. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/mandiblemaxillacalcifyingodontogenic.html. Accessed March 4th, 2021.
Definition / general
- WHO defines entity as a benign cystic neoplasm of odontogenic origin, characterized by an ameloblastoma-like epithelium with ghost cells that may calcify
- Originally described by Gorlin and colleagues in 1962 as a possible oral analogue to pilomatrixoma of skin, owing to the presence of ghost cell keratinization in both lesions
Terminology
- Calcifying cystic odontogenic tumor (CCOT) is preferred 2005 WHO terminology
- However, also called Gorlin cyst, calcifying odontogenic cyst (COC) , keratinizing and calcifying odontogenic cyst
- Peripheral COC: in gingival tissues, ie: extra-osseous
- Central COC: within bone of jaws, ie: intra-osseous.
- The following discussion pertains only to central COC
- Several histopathologic classifications have been delineated, with nearly a dozen potential subgroupings
- From a practical standpoint, a spectrum of histopathologic patterns exists, ranging from a benign lesion that is primarily cystic, a benign lesion with a solid pattern of growth to a rare tumor with features of carcinoma
- Solid lesions may be more aptly designated Dentinogenic Ghost cell tumors
- Simple cystic, non-proliferative: single chambered cyst lined by squamous or stellate reticulumlike cells with a degree of palisading of the basal cells; ghost cells present, eosinophilic dentinoid is variably present and often juxtaepithelial, but no other hard tissues seen
- Cystic, proliferative/ameloblastomatous: in addition to simple cystic features, there is proliferation of ameloblastoma-like cords, islands, and sheets of epithelium with palisading of basal cell nuclei with reverse polarization within the wall; odontogenic epithelial proliferations that can superficially resemble ameloblastoma extend into the lumen as well as the connective tissue wall of the lesion
- Odontoma-associated: Odontoma-like tissues are seen in the wall of the lesion; when associated with an odontoma, patient age tends to be younger, with mean age 17 years
- Dentinogenic ghost cell tumor:
- Also called epithelial odontogenic ghost cell tumor, odontogenic ghost cell tumor
- Similar components to COC, but without a cystic structure and with a neoplastic growth pattern (Odontogenic ghost cell tumor)
- For a detailed classification of ghost cell tumors, refer to Table 2 of the International collaborative study on ghost cell odontogenic tumours (J Oral Pathol Med 2008;37:302)
- See also Am J Surg Pathol 2003;27:372, Oral Surg Oral Med Oral Pathol 1991;72:56
Epidemiology
- 6% of central odontogenic tumors (J Oral Maxillofac Surg 2006;64:1343)
- Mean age 31 years, range 5 - 92 years
- No consistent gender predominance (studies report both)
- 85% have cystic component, 15% have solid pattern reminiscent of neoplastic process
Sites
- Usually anterior regions of jaws, incisor cuspid region
Pathophysiology
- Controversial whether a developmental cyst or a true neoplasm
- In 1992, WHO classified this lesion as odontogenic tumor but continued to use the term calcifying odontogenic cyst
- In 2005, WHO redesignated the lesion as calcifying cystic odontogenic tumor
- Although the condition is often described as being cystic (> 85% of the cases), a significant percentage of calcifying odontogenic cysts grow as more solid, seemingly neoplastic proliferations, and the term dentinogenic ghost cell tumor has been used to describe these lesions
- Even the cystic lesion has apparent neoplastic potential but most appear to be non-neoplastic
Clinical features
- Either none (incidental radiographic finding of painless bony expansile remodeling) or pain
- May be associated with other odontogenic pathology, most commonly odontoma
Radiology description
- Usually unicystic, well defined radiolucency with focal opacification (Br J Radiol 2012;85:548) but 10 - 25% are multilocular
- Scattered radiopacities in 1/3 to 1/2
- 1/3 associated with impacted tooth
- Usually 2 - 4 cm, but up to 12 cm
- Adjacent tooth root resorption or root divergence may occur (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101:356)
Prognostic factors
- Prognosis excellent, few recurrences (< 5%) documented
- Rarely malignant transformation (Case Rep Pathol 2013;2013:853095, Korean J Pathol 2012;46:478, Ann Diagn Pathol 2009;13:394)
Case reports
- 15 year old Japanese boy with pigmented calcifying cystic odontogenic tumor associated with compound odontoma (Head Face Med 2007;3:35)
- 23 year old man with ameloblastic fibro-odontoma arising from a calcifying odontogenic cyst (Bull Tokyo Dent Coll 2001;42:51)
- 24 year old woman with calcifying odontogenic cyst associated with an orthokeratinized odontogenic cyst (Head Neck Pathol 2008;2:324)
- Calcifying odontogenic cyst with odontogenic keratocyst (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;109:e40)
- Hybrid odontogenic tumor with features of ameloblastic fibro-odontoma, calcifying odontogenic cyst, and adenomatoid odontogenic tumor (J Oral Maxillofac Surg 2010;68:470)
- Calcifying odontogenic cyst with ameloblastic fibroma (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;98:451)
Treatment
- Treated by enucleation and curettage
- For combined lesions, treat according to characteristics of more aggressive lesion
Clinical images
Gross description
- Unicystic, often 2 cm or less; no solid areas
Microscopic (histologic) description
- Well defined layer of palisading basal cells, loosely arranged suprabasal epithelial cells resembling stellate reticulum similar to ameloblastoma
- Unlike ameloblastoma, variable numbers of lesional epithelial cells undergo ghost cell change in suprabasilar epithelium
- Epithelium also contains pale, eosinophilic ghost cells that may keratinize or calcify; dentin may be laid down next to basal cells; variable foreign body reaction
- Ghost cells: altered epithelial cells with preservation of basic cell outline, eosinophilic cytoplasm, but loss of nucleus
- Can exhibit dystrophic calcifications
- Ghost cell change may be due to coagulative necrosis, accumulation of enamel protein, aberrant keratinization of odontogenic epithelium
- Dentinoid:
- Varying amounts of a paucicellular, eosinophilic calcified material considered to represent dysplastic dentin
- May be present adjacent to epithelial component
- Probably due to inductive effect by odontogenic epithelium on adjacent mesenchymal tissue
Microscopic (histologic) images
Contributed by Kelly R. Magliocca, D.D.S., M.P.H.
Images hosted on other servers:
Cytology description
- Numerous polyhedral epithelial cells and occasional columnar cells with calcification and Congo red-negative extracellular homogenous material in background (Acta Cytol 2009;53:460)
Positive stains
- Cytokeratins 7, 8, 14 and 19 (J Oral Pathol Med 2003;32:163)
- Beta-catenin (APMIS 2008;116:206, Pathol Int 2006;56:732, Am J Clin Pathol 2003;120:732, Am J Pathol 2003;163:1707)
- Amelogenesis related proteins (J Oral Pathol Med 2012;41:272)
- MMPs: Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2011;112:609
- Hard alpha-keratins (Am J Clin Pathol 2005;123:376)
- Podoplanin (J Oral Sci 2012;54:165)
Negative stains
- Ki67 lower in COC than in other odontogenic tumors with ghost cells and solid growth pattern (Oral Oncol 2009;45:515)
Electron microscopy description
- Basal and suprabasilar cells contain tonofilaments and organelles
- Ghost cells contain coarse bundles of tonofilaments intermingled with dilated membranous organelles (Med Electron Microsc 2002;35:109, Oral Surg Oral Med Oral Pathol 1975;39:769)
Molecular / cytogenetics description
- Aberrations of Wnt signaling pathway with beta-catenin overexpression (APMIS 2008;116:206)
Differential diagnosis
- Central jaw lesion with basal palisading of epithelium
- COC
- COC in combination with second tumor
- Ameloblastoma
- Keratocystic odontogenic tumor
- Dentinogenic ghost cell tumor/carcinoma
- Odontogenic cyst or tumor, NOS
- Ghost cells identified, unknown anatomic location:
- COC
- Dentinogenic ghost cell tumor
- Ghost cell odontogenic carcinoma: infiltrative and exhibits mitotic activity, nuclear atypia, and/or necrosis.
- Odontoma
- Ameloblastic fibro-odontoma
- Pilomatrixoma
- Matrical carcinoma
- Craniopharyngioma