Calcifying Epithelial Odontogenic Tumor
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The calcifying epithelial odontogenic tumor (CEOT), also known as a Pindborg tumor, is an odontogenic
tumor A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...
first recognized by the Danish pathologist Jens Jørgen Pindborg in 1955. It was previously described as an ''adenoid adamantoblastoma'', ''unusual
ameloblastoma Ameloblastoma is a rare, benign or cancerous tumor of odontogenic epithelium (ameloblasts, or outside portion, of the teeth during development) much more commonly appearing in the lower jaw than the upper jaw. It was recognized in 1827 by Cusa ...
'' and a ''cystic odontoma''. Like other odontogenic neoplasms, it is thought to arise from the epithelial element of the enamel origin. It is a typically benign and slow growing, but invasive neoplasm.


Types

Intraosseous tumors (tumors within the bone) are more common (94%) than extraosseous tumors (6%). It is more common in the posterior
mandible In anatomy, the mandible, lower jaw or jawbone is the largest, strongest and lowest bone in the human facial skeleton. It forms the lower jaw and holds the lower teeth in place. The mandible sits beneath the maxilla. It is the only movable bon ...
of
adult An adult is a human or other animal that has reached full growth. In human context, the term ''adult'' has meanings associated with social and legal concepts. In contrast to a "minor", a legal adult is a person who has attained the age of ...
s, typically in the fourth to fifth decades. There may be a painless swelling, and it is often concurrent with an impacted
tooth A tooth ( : teeth) is a hard, calcified structure found in the jaws (or mouths) of many vertebrates and used to break down food. Some animals, particularly carnivores and omnivores, also use teeth to help with capturing or wounding prey, t ...
. On
radiograph Radiography is an imaging technique using X-rays, gamma rays, or similar ionizing radiation and non-ionizing radiation to view the internal form of an object. Applications of radiography include medical radiography ("diagnostic" and "therapeuti ...
s, it appears as a radiolucency (dark area) and is known for sometimes having small radiopacities (white areas) within it. In those instances, it is described as having a "driven-snow" appearance. Microscopically, there are deposits of
amyloid Amyloids are aggregates of proteins characterised by a fibrillar morphology of 7–13 nm in diameter, a beta sheet (β-sheet) secondary structure (known as cross-β) and ability to be stained by particular dyes, such as Congo red. In the hu ...
-like material. The underlying nature of the amyloid-like material is still unresolved.


Clinical features

Clinically it has two types, the central and the peripheral. The central type of the CEOT occurs in individuals ranging in age from 20 to 60 years. Two-thirds of the lesions are in jaws, more commonly in the molar area with a tendency to occur in the pre-molar areas. It appears clinically to be a slowly enlarging painless mass. In the maxilla it can cause proptosis, epistaxis and nasal airway obstruction. The peripheral type is commonly found in the anterior region of the maxilla and occurs as a soft tissue swelling.


Histology

Histopathology will reveal prominent intercellular bridges and nuclear changes such as pleomorphism, hyperchromatism and prominent nucleoli. The mitotic figures are rare. Spread throughout the epithelium and connective tissue are spherical amorphous calcifications. Variants include the clear cell CCEOT; whose cells have clear cytoplasms. The incidence is low, with 15 cases of CCEOT reported. However, another study has indicated that approximately 8% of CEOTs contain clear cells. There is one case reported in the literature of a 15-year-old white male with a true cystic variant of CEOT.


Recurrence

The recurrence rate is 10-15%. Franklin and Pindborg reported a recurrence rate of 14%. It is considered to have a recurrence rate much lower than that of
ameloblastoma Ameloblastoma is a rare, benign or cancerous tumor of odontogenic epithelium (ameloblasts, or outside portion, of the teeth during development) much more commonly appearing in the lower jaw than the upper jaw. It was recognized in 1827 by Cusa ...
. Malignant metastasis has been reported.


See also

* ODAM (gene) *
Proteopathy In medicine, proteinopathy (; 'pref''. protein -pathy 'suff''. disease proteinopathies ''pl''.; proteinopathic ''adj''), or proteopathy, protein conformational disorder, or protein misfolding disease refers to a class of diseases in which certa ...
*
Tooth development Tooth development or odontogenesis is the complex process by which teeth form from embryonic cells, grow, and erupt into the mouth. For human teeth to have a healthy oral environment, all parts of the tooth must develop during appropriate stag ...


References

{{reflist *Zahid, Arsalan from Contemporary Oral and Maxillofacial Pathology *Kahn, Michael A. Basic Oral and Maxillofacial Pathology. Volume 1. 2001. Oral neoplasia