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Odontogenic Tumors

Odontogenic Tumors

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Published by Dr. Hesham Zaatar

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Published by: Dr. Hesham Zaatar on Apr 02, 2011
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02/05/2013

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O
DONTOGENIC
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UMORS
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UMORSINTHEJAWSTHATARISEFROMODONTO
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genic (tooth forming) tissues are referred to asodontogenic tumors. But what are “odontogenictissues” and how do you get tumors from them inadults long after odontogenesis has ceased? If youremember the embryology and histology of tooth for-mation, you may skip the next three paragraphs andgo directly to the tumors starting with “ameloblas-toma”.Recall that two types of embryonic tissues con-tribute to the formation of a tooth. Early in embryoge-nesis, future dental pulp cells (primitive ectomes-enchyme) migrate from the neural crest to the jawsand settle out in areas where teeth are to be formed.They signal the overlying ectoderm (epithelium) tosend down a cord of cells (the dental lamina) whichwill become the enamel organ. It is the inner layer of the enamel organ that will become ameloblasts thatsecrete enamel matrix. (Amelogenin is the main pro-tein found in enamel matrix. The gene that encodesthis protein is on the X chromosome.)After the arrival of the enamel organ, the awaitingectomesenchymal cells turn into dental pulp cells. Theouter cells of the pulp that are adjacent to theameloblasts differentiate into odontoblasts and makedentin matrix whereupon the ameloblasts make enam-el matrix. After the crown forms, the inner and outerlayers of the enamel organ squeeze out the two middlelayers (stratum intermedium and stellate reticulum)and grow downward as a cylinder shaped structure(Hertwig’s root sheath) that outlines the root. Soremember, a tooth is formed from two types of embry-onic tissues, ectoderm and ectomesenchyme. It is oneof the many wonders of embryogenesis that these tis-sues are genetically programmed so that they form asingle rooted tooth in the front, a double rooted toothin the midjaw, and double and triple rooted teeth inthe posterior jaws. How do they know?The point in telling you this is to refresh yourmemory about terminology and to tell you that all of these tissues do not undergo apoptosis when the job isdone. Remnants of odontogenic epithelium remain inthe periodontal ligament and gingiva forever. In thegingiva, they are called epithelial rests of Serres andin the periodontal ligament they are known as the restsof Malassez. It is not known if remnants of ectomes-enchyme remain because it is indistinguishable fromsurrounding mesodermal tissues. It is from the epithe-lial rests (Serres and Malassez) that arise odontogenictumors in adults. There is a third source, the reducedenamel epithelium that envelopes the crown of unerupted teeth. Now the tumors.
 AMELOBLASTOMA
Ameloblastoma is a tumor in which the tumor cellsform caricatures of the enamel organ and some of them resemble ameloblasts. However, they are inca-pable of making enamel matrix. This tumor occurschiefly in middle age people long after odontogenesishas ceased. Presumably a carcinogen converts a cell inone of the epithelial rests to become a tumor cell. Assuch, it starts to divide endlessly to form a tumor.Remember the axiom, tumor cells tend to resemblethe tissues from which they arise. Since cells in restsof Serres and Malassez were at one time capable of becoming ameloblasts, it should not come as a sur-prise that tumor cells resemble ameloblasts.What are the main points about this tumor? It isunencapsulated and infiltrates surrounding bone mar-row. Even though they are locally infiltrative, they donot metastasize. They may occur in any part of both jaws but most are in the middle and posterior regionsof the mandible. Ameloblastomas are always purelyradiolucent and may be unilocular but frequentlybecome multilocular as they increase in size.If not found and treated early, they will expand the jaw.Histologically, the tumor cells appear as islands of odontogenic epithelium in a stroma of fibrous connec-tive tissue.The cells in the center of the islands resemble stel-late reticulum but may undergo squamous metaplasia
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 Ameloblastoma,medium powermicroscopy. Islands of odontogenic epitheliumin a stroma of fibrousconnective tissue.
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 Ameloblastoma: a multilocular radiolucentlesion in the body and ramus of the mandible in a29 year old female.
ODONTOGENIC TUMORS:THE SHORT VERSION
 
to become keratinizing squamous epithelium. Thecells at the periphery of the epithelial islands are tallcolumnar cells that rest on a basement membrane sep-arating them from the surrounding stroma.The nuclei of the tall columnar cells (ameloblasts)are typically found at the end of the cell most distantfrom the basement membrane, a feature referred to as“reverse nuclear polarity”.The treatment of ameloblastoma is surgical exci-sion. This may require removal of a large segment of the jaw. Simple curettage is usually met with recur-rence.Ameloblastomas that arise from rests of Malassezoccur as intrabony tumors. Rarely, ameloblastomaarises from rests of Serres. Here they grow as extra-bony soft tissue tumors in the gingiva (Figure 4).Additionally, the reduced enamel epitheliumaround the crown of unerupted teeth may turn intoameloblastoma in which case it radiographically mim-ics a dentigerous cyst (Figure 5).This type of tumor has been referred to as unicysticameloblastoma or simply as cystic ameloblastoma andhas a much better prognosis.
 ADENOMATOID ODONTOGENIC TUMOR(AOT)
This is a tumor mostly of teenagers. It occurs in themiddle and anterior portions of the jaws in contrast toameloblastoma which is found mostly in the posteriorsegment. Two-thirds occur in the maxilla and it ismore common in females. This tumor is encapsulatedand is treated by curettage with a recurrence rateapproaching zero.The radiographic appearance is a unilocular radi-olucency, often around the crown of an uneruptedtooth in which case they resemble a dentigeous cyst(Figure 6).Histologic examination reveals a thick capsule of fibrous connective tissue. The tumor fills the centralcavity, there is little stroma. Tumor cells frequentlyform ball-like structures referred to as “rosettes”(Figure 7).
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 Adenomatoidodontogenic tumor, medi-um power. Note how thetumor cells form balls of cells that are calledrosettes.
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 A peripheralameloblastoma. This tu-mor arises from rests of Serres or alternately frombasal epithelial cells inthe gingiva in a manneranalagous to cutaneousbasal cell carcinoma.
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UMORS
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 A cystic ameloblastoma associatedwith an unerupted and displaced molar toothin a child. Note the hint at compartmental-ization (multilocularity). It resembles an ordi-nary dentigerous cyst except that dentiger-ous cysts are ordinarily unilocular.
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 Ameloblastoma, high power micro-scopy. The center and left are odontogenicepithelium. The fibrous connective tissue stro-ma is right and bottom. Notice the loose stel-late reticulum-like appearance in the middle of the epithelial island and the tall columnar cellswith reverse nuclear polarity at the outer edgeof the epithelium (see arrow).
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 Adenomatoidodontogenic tumor (AOT)associated with anunerupted tooth #11. Itresembles a dentigerouscyst. Some AOTs pro-duce calcifications thatmay appear as “snow-flake” densities.
 
Another identifying feature is the presence of duct-like structures (Figure 8).Small calcifications may be found in the tumor. If they are present in sufficient size and number, theymay show on the radiograph as a “snow-flake” pat-tern. A homogeneous, eosinophilic and amorphousmaterial may occasionally be found in AOT. Its identi-ty is disputed. Some consider it a peculiar form of enamel matrix, others claim that it is a dentin-likematerial. This is of no clinical importance but doesraise questions if this is a pure epithelial tumor (enam-el matrix) or has an ectomesenchymal component(dentin matrix).
CALCIFYING EPITHELIAL ODONTOGENIC TUMOR (CEOT, Pindborg tumor)
This tumor is so rare that it deserves little discus-sion. In 1958, Dr. J.J. Pindborg of Denmark describedfour cases of an unusual odontogenic tumor that stillbears his name. This is the most “unodontogenic”tumor of the group, the tumor cells do not resembleodontogenic tissue. Tumor cells are squamous-like,they even have desmosomal attachments. There oftenis variation in nuclear size, shape and staining intensi-ty—features that are often associated with malignancy(Figure 10). This tumor forms an amorphous materialthat is said to be amyloid or amyloid-like (Figure 11).Whatever it is, it calcifies in a concentrically lamellat-ed “tree-ring” pattern known as Liesegang calcifica-tions. This explains the name of calcifying epithelialodontogenic tumor.CEOT lacks a capsule but apparently does not infil-trate as deeply into surrounding tissues as doesameloblastoma. Excision with a small margin of sur-rounding bone is usually curative.Radiographically, this tumor may appear as a radi-olucent tumor but if sufficient calcifications are pres-ent, it becomes dense (Figure 9).NOTE: We have skipped malignant ameloblas-toma, ameloblastic carcinoma, clear cell odontogeniccarcinoma and odontoameloblastoma and squamousodontogenic tumors because they are too rare. Theyare covered in your text.
 AMELOBLASTIC FIBROMA
This is a tumor of childhood, the typical patient isabout 12 –14 years old, seldom is it seen beyond age20. The posterior segment of the mandible is the mostcommon location. Local swelling or failure of teeth toerupt on time or in proper alignment may call atten-tion to the tumor. Ameloblastic fibromas are purelyradiolucent.
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Calcifications(extreme left) in a CEOTaccount for the densitynoted on radiographs.These are thought to becalcified amyloid oramyloid-like material,some of which can beseen right of center as apale eosinophilic globule(arrow).
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CEOT, highpower view. The tumorcells resemble squamousepithelium more thanodontogenic epithelium.There is some variationin nuclear size andshape, but this is not amalignant tumor.
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Calcifying epithe-lial odontogenic tumor inthe body of themandible. It appears asa radiolucent lesion withsmokey dense areas.
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 Adenomatoidodontogenic tumor. Notethe duct-like structures(see arrow)
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 Ameloblasticfibroma in and aroundthe crowns of lowermolar teeth. It is subtle,the second deciduousmolar tooth has been tilt-ed downward by thetumor. Patient was a 5year old boy.

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